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JlIUf1IU1 of PedisIric ~ .p0;i1 B. .':103-107 C 2000 Lippincott Williams & Wilkins, Inl:., Philade1phl3

Wallace B. Lehman, M.D.,* Dan Atar, M.D., t David S. Feldman,M.D.,* JonathanC. Gordon, M.D.,;!: and Alfr~ D. Grant, M.D.§

.DepartmenJ ofPediotric Orthopaedic SurgezyJ Center for Pediatric Orthopaedic Surgery, Hospitalfor Joint DiseasesCerLierfor Children,NYU Sdwol of Medicine, New Yoric, USA; tDepartment of Orthopaedic3, Soroka Medical

Center, Ben-Gurion University of the Negeu, Beer-SheIXI, Israel; tHahnemann Medical School, Philadelphia,P~nnsy~ia, USA; and §Eliy Hammennan Center for Trtatmento! Neuromuscular Disorders, Ho.spital for Joint

Diseases Center for Children, N~ Yo'*. USA

tion that early primary amputation with an appropriateprosthesis should be considered, and that the fjnal evalua-tion should not be based on obtaining bone union, but onthe level of function of the lower extremity. Key Word~:Pseudoartbrosis-Tibia-Surgica1 treatment.

t Summary: CongeniW pseudoarthrosis of the tibil! remains.one of the most difficult conditions to treat in orthopedicI surgery. Seven cases were treated in our hospital by dif-i fereat methods, Three out of sev~n patients were healed,l two of these reuactured. At follow-up, the success rate, was 14% (one out of seven cases). It is our reco1'I1mcnda-

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I.was 2.5 years (range, 2-5 years). The first surgicalprocedures were: resection of pseudoarthrosis, plat-ing and bone graft (two cases), resection and bonegraft (two cases), resection and intramedullary rodand bone gr~ (one ca$e), Ilizarov distraction-com-pression (one case), and resection plus free vascular-izedbone gruft (one case) <Table 1).

Congenital pseudoarthrosis of the tibi3 is one ofthe most difficult conditions to treat in orthopedics(11,21). Previously, operative treatment h~ beenadvised for patients older th~ 4 years of age(1,9,18,21). More recently, the advantages of an earlyoperation and a rapid reoperation, if bony union wasnot achieved, have been emphasized (4,.14,15,20,21).Various surgical techniques have been used eithersingularly or in combination, including bone graft-ing, internal and external fuation, electrical stimula-tion, free vascularized grafts and amputation afterother procedures fail (4-7,9,10,17,20,22,23,25,30). Inthis article, we present our experience with severalselect surgical techniques and further update th~guidelines for surgical treatment.

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RESULTS

Thrce patients underwent four to six additionalsurgical procedures after the first procedure hadfailed. Three patients had two to three repeated~urgeries. Only one patient had one operation. Ad-ditional procedures included: rush rod, re-plating,llizarov, free vascularized bone graft, intramedullarynail, re-bone graft, and electrical. stimulation. Fol-low.up ranged from 1 year to 8 years. At follow-up,union wc)s achieved in only one patient (number 6).Howevcr, the tibia Was short and in this patient, the~nkl.e was in valgus. In patients 2 aI:1d 4, union wasachicvcd with the nizarov apparatus, but refractureoccurred within 1 year after Union was achieved andthe tibia was not united at foUow-up (Figs. 1-6).Patient 2 underwent a below-knee amputation 2years later.

MATERIALS AND Ml!.lHODS

Seven patients (four boys, three girls) with con-genital pseudoarthrosis of the tibia (Boyd type ll-high risk) had been treated during the period1987-1992. In each case, the pseudo~rthrosis w~snot related to neurofibromatosis. All patients hadSustained fractures of the tibia during their first tosecond ycar of life and were casted for extendedperiods of time. The average age at initial surgery

DISCUSSIONB Address correspondence and rep..int re:quests to Dr W&nace301~hD1an. Pediatric Orthopaedics, Hospital For Joint Diseases,

East 17th Street, New York, NY }0003, USA. A fracture and pscudoartllrosis are present at

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FIG. 4. X-rays 6 months later: bOny union was ach~.

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107CONGENITAL PSEUDOARTHROSIS OF THE TIBIA

ytar after the pseudoarthrosis was healed.. Up to30% to 60% of patients may still end up requiringan amputation, usually after multiple operativeprocedures, whe~ signifi~ant deformity ~ti11 ~stsand when there IS excessive leg length Qtsctepancy(3,4,11,14.17,30).

In the cases described in this article. severalsurgi-cal m~thods were applied. None seemed to prevail.However, the trend in the literature today in es.tablished pseudo~rthrosis of the tibia is to excise thepseudoarthrosis, to insert an I.M. rod (trans-Qilcaneal) and to perform a bone graft. In the f~iledcases and in the older age groups, resection of thepseudoarthrosis and application of the compression-distraction Ilizarov apparatus with proxiroal cortico-tomy seems to be the treatment of choice (20).However, it is the authors' belief that primary ampu-tation at an early age 'with an appropriate prosthesisis a better choice.

We do not think that treatnlent of congenitalpseudoarthrosis should be based on obtaining a bonyunion. Instead, it should be based on obtaining thebest and most useful leg at maturity with the leastamount of permanent trauma to the patient's legand general development, including psychologicaldevelopment. This series of ~es used many treat-ment xnodaJities, including the llizarov, intra-medullary nailing with grafting, and v8$cular bonegrafts. Bony union was obtained only to witness laterrefractures that required additional bracing andsurgery, and sometimes amputation with need forprosthesis. Is a child who, at 15 years of age hasundergone multiple operative procedures and has anugly, deformed, braced leg, anq who is unable toparticipate in many desired activities, what we want?Or is the patient with an early-amputated leg and agood prosthesis who can participate in most activi-ties the better outcome? Evaluating the treatment ofpseudoarthrosis on the basis of methods of obtainingbony union is probably not in the best interest of thepatient. The quality of function of a mature child'sleg alter multiple $urgical procedures versus ampu-tation should be the criterion that is used for treat-ing congenital pseudoarthrosis (12,27).

6. Bassett CA, Caulo N, Kort J. OJngepit31 pscudoarlhrosis ofd1e tibia: treatment with pulsing electromagnetic fields. ClinOrthop 1981;154:136.

7. B<JYd HB, Ss.ge FP. Congenital ~doarthrosis of the ~ibia.J Bone Joint Swg 1958;40A:1245-1210.

8. Crossett 1.S, BeatyJH. BetzkR ~ngenit81 pseudoarthrosisof the tibia. Clin Orrhop 1989;245:16-.,8.

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.11. Hs.rqinge K. Congenital anterior bowing of d1e tibia. Ann RCdl SulK EngI1972;51:17-30.

12. KamiLA, H~ideri NF, Halliday SE. Smitherman TB. John-son CE II. Gait analysis and muscle strength in childrl:n withcongenital ~udoarthrosi5 of thc ~ibi3: the effect of treat.ment. JPediatr Orthop 1998;1.8:381-6. .

13. Uoyd-koberts G<:;, Shaw NE. The prevention of pseu-doarthrmis in congenital kyphQSis of the tibia) Bone Joint$wg 1969;518:100-105.

14. Massennan Rl., Peterson HA, Biano AJ. Congenital pseu-doarthrosis of the tibia: a review ofthc literature and 52cases from the Mayo Clinic CIin Orthop 1974;99:140-~.

15. McElhannQn PM. Congenit~ pseudoarthrosis of the I1bia.South Met/ J 1975;68:324.

16. McFarland B. Pseudoarthrosis of the tibia in childhood. JBon" Joint Surg 1951;33B:26.

17. Momssy RT, Riseborough EJ, Hall JE. Congenital pseu-doarthr0si5 of the tibia: LO:~ teIUl follow up of 40 cases. JBone Joint $u!6' 1979;61B:246-247.

18. Murray 1m, Lovell WW. Co~enitaJ peeudoarthrosis of d1etibia. J Bone 1oint Su!6' 1981;63B:367-375.

19. Murray HR, Lovell WW. Congenital pseudoarthrosis of thetibia. Clift Orthop 198Z;166:14-20.

20. Paley p, Catagni M. Argnani F. Prevot J, Bell D, AnnstrongP. Trcatm~tof congenital pseudoarthrosis of the tibia usinsthe Uizarov technique. Clin Orrhop 1992;280:81-93.

21. Paterson D. Congenital pseudoarthrosis of the tibia: anoverview. Clin Orthop 1989;247:44-54.

22. php kWH, Levack B. Satku K. Patradul A. Prce vascular-ized fibula graft in the treatment of congenjtal pseu-doarthrosis of the tibia. J Bone Joint SU/81985;678:64-70.

23. Plawecki F. Carpentier E, La=mbes P. Treatment of con-genital pscudoarthosis of the tibia by the l\izarov meth9d. JPet/iotr Orthop 1990;10:186-90.

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25. Sim(1nis RD, ShiraliHR, Mayou B. Free vascularized fibulargrafts for congenital PS"udoarthrosis of the tibia. j BoneJoint Sutg 1991;73B:211-21S.

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27. Traub JA, O'Connor W, Masso PD. Congenital pseu-doarthro5i$ of the tibia: a retrospective review. J PediatrOrrhop 1999;19:735-8.

28. Uchida Y. Kpjima T, Sugioka Y. Vascularized fibular graftfor congenital pseudoarthrosis of the tibia: Jonglenn results.J Bone Joint Suit 1991;738:846-50.

29. Umber 1S, Moss SW, Coleman SS. Surgical trealment ofcongenital pseudoarthrosis of Ibe tibia. Clin Onhop1982;166:28-33.

30. Ness CP Yan. Congenital pseudoarthrosis of the leg. J BoneJoint Sutg 1966;48A:1461-1483.

31. Weiland AJ, Weiss AC, Moore JR, Tolo VT. Vascularizcdfibular grafts in the treatment pf congenital pseudoarrhosisof the tibia. J Bone JointSUIg 1990;72A:654-62.

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