Article ID: WMC005569 ISSN 2046-1690

Congenital Vertical Talus - Etiology, Pathogenesisand Current Updates in Management.Peer review status:No

Corresponding Author:Dr. Karthick S.r,Senior Resident, Orthopaedics, PGIMER, Chandigarh,India, H BLOCK 105, MARRIED DOCTORS HOSTEL,,PGIMER, CHANDIGARH, 160012 - India

Submitting Author:Dr. Karthick S.r,Senior Resident, Orthopaedics, PGIMER, Chandigarh,India, H BLOCK 105, MARRIED DOCTORS HOSTEL,,PGIMER, CHANDIGARH, 160012 - India

Other Authors:Dr. Pulak Vatsya,Senior Resident, Orthopaedics, PGIMER, Chandigarh,India, PGIMER, CHANDIGARH, 160012 - India

Dr. Nirmal Raj Gopinathan,Additional Professor, Orthopaedics, PGIMER, Chandigarh,India, PGIMER, CHANDIGARH, 160012 - India

Dr. Prashant Sharma,Fellow, Orthopaedics, PGIMER, Chandigarh,India, PGIMER, CHANDIGARH\n, 160012 - India

Article ID: WMC005569

Article Type: Review articles

Submitted on:12-May-2019, 01:03:44 PM GMT Published on: 20-May-2019, 08:33:52 AM GMT

Article URL: http://www.webmedcentral.com/article_view/5569

Subject Categories:ORTHOPAEDICS

Keywords:Congenital Vertical Talus ; Current concepts

How to cite the article:S.r K, Vatsya P, Gopinathan N, Sharma P. Congenital Vertical Talus - Etiology,Pathogenesis and Current Updates in Management.. WebmedCentral ORTHOPAEDICS2019;10(5):WMC005569

Copyright: This is an open-access article distributed under the terms of the Creative Commons AttributionLicense(CC-BY), which permits unrestricted use, distribution, and reproduction in any medium, provided theoriginal author and source are credited.

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No source of funding

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Conflict of interest : Nil

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Congenital Vertical Talus - Etiology, Pathogenesisand Current Updates in Management.Author(s): S.r K, Vatsya P, Gopinathan N, Sharma P

ABSTRACT

Congenital Verical Talus (CVT) is a rare foot deformitywhich is most often unrecognised/ misdiagnosed atbirth. Inadequate management leads to abnormal gait,shoe wear problems, callosities, pain and functionallimitations. Etiology is heterogenous - genetic disorderassociated, neuro muscular or idiopathic. Restorationof normal alignment of bones of foot and weightbearing capacity of first ray are primarily aims oftreatment. Depending on severity and rigidity ofdeformities multiple extensive as well as minimalinvasive surgical procedures have been described forCVT. In recent times, management has drasticallychanged from earlier methods of extensive soft-tissuerelease procedures to the serial manipulation, castingand minimal invasive surgery for Talo navicular jointreduction.

INTRODUCTION

Congenital Vertical Talus, being first described in 1914by Henke, is one of the causes of congenital rigidflatfoot, inadequate management of which leads toabnormal gait, shoe wear problems, callosities, painand functional limitations. The rocker-bottomappearance of the medial border of foot, which isclassical to this condition, is mostly not apparent atbirth, which can lead to the clinician missing thediagnosis, further adding to the problem. The primarypathology in this condition is believed to be the dorsaldislocation of navicular over the talar head, leading tothe almost vertical orientation of the talus, a dislocatedtalonavicular joint, a hindfoot in valgus and equinusand an abducted forefoot. This has been managedclassically by extensive surgeries, with soft tissuestripping and release of associated ligamentouscontractures and reduction of talonavicularjoints.[1][2][3] This is followed by a dreaded course ofcontractures and suboptimal outcomes for thesepatients. Dobbs et al,[4] in recent times havepresented their management protocol, based oncasting and soft tissue stretching, similar to Ponsetiphilosophy used in clubfoot, followed by minimalinvasive talonavicular reduction and tendo-achillesrelease, with a good, supple, plantigrade feet on

short-term follow ups, giving hope of a bettermanagement protocol for these patients. This review,gives an overview of current understanding ofconcepts of CVT and the recent advances inmanagement protocols.

EPIDEMIOLOGY ANDETIOLOGY

EPIDEMIOLOGY

The reported incidence at present is 1 in 10,000, afigure which has continued since the past 20 years,[5]signifying the lack of adequate data and large samplebased studies to update the epidemiological datalike laterality, ethnicity or other risk factorsassociated with this disease. It is considered to havean equal incidence in males and females. Theindecisive epidemiological data about this conditionputs light on the fact that this condition is missed andunder-reported. A wider population based andmulticentre data would let us know more about thetrend, associated risks and when to suspect thiscondition, which would eventually improve theoutcomes.

 ETIOLOGY

Half of these cases are considered as isolated oridiopathic in origin, where-as the other half areassociated with neuromuscular[6] or geneticdisorders[7]. Cause is still not identified, althoughsome evidence indicates, that the deformity istransmitted as an autosomal dominant trait, withincomplete penetrance. Mutations in homeoboxtranscript ion factor and cart i lage-derivedmorphogenetic protein-1, are indicated as contributorsin some of the familial cases.[8] Although, no singlegene can be pointed out and the development of thisdisorder is essentially heterogenous.

Most commonly associated neurological conditions arearthrogryposis and myelomeningocele, whereas mostcommon genetic conditions found to be associated areaneuploidy of chromosome 13, 15 or 18. Otherneurological conditions are disatometamyelia, lipomao f cauda equ ina , sac ra l agenes i s andneurofibromatosis to name a few.[6] NeurologicalCVTs are found to be of the more rigid. The imbalance

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in muscle strength in neurological cases has beenpostulated to be one of the causes. This is justified bythe weak posterior tibialis and the stronger dorsiflexorsof ankle, with foot intrinsic muscle contractures orweakness playing a role, as seen in CVT withmyelomeningocele. This is further supported by thehigh number of abnormal muscle biopsies obtained inthese patients. A number of syndromes namelyDeBarry, Costello, Rasmussen as well as split handand foot syndromes have been found to be associatedwith CVT in many cases.Acquired deformities leadingto CVT are cerebral palsy, polio, overcorrectedclubfoot and spinal muscular atrophy to name a few.Â

LITERATURE SEARCH

Congenital Vertical Talus is a much talked about topicin the recent times, especially since Dobbs et al. havepresented a novel method of minimal invasivemanagement, based on casting principles of Ponseti,which have yielded spectacular results.

A primary PubMed search was done on 1st May 2019with keywords; (“congenital, Vertical, Talus"[MeSHTerms] OR ("Congenital"[All Fields] AND "Vertical"[AllFields] AND "Talus"[All Fields]).This yielded 2635results. On adding filters – “Review and 10years―, the number was bought down to just 155articles. Of these only 2 articles, had “CongenitalVertical Talus― in their title. On further adding“OR AND ("Paediatric"[MeSH Terms]― narroweddown our search to only 9 review articles. All thesearticles were primarily reviews about paediatric rigidflatfoot, one of the causes of which is congenitalvertical talus.

On using (paediatric rigid flatfoot AND Review [ptyp]AND "last 10 years"[PDat]), gave us only 29 hits, noneof which had congenital vertical talus in their title.

PATHOANATOMY

Surgical and autopsy findings have constantly addedto understanding of anatomy of soft tissue and bonesin a foot with vertical talus.[1][9] The hindfoot is inequinus and valgus due to contracture of Achillestendon and posterolateral ankle and subtalar jointcapsule. Midfoot and forefoot are dorsiflexed andabducted relative to hindfoot secondary tocontractures of tibialis anterior, EDL, EHB, peroneustertius and EHL and dorsal aspect of talonavicularcapsule. The navicular is dorsally and laterallydislocated on the head of talus resulting in ahypoplastic and wedge-shaped navicular. Talar head

and neck are abnormal in shape and orientation, aflattened head with a medial orientation from themidline. The position of the talus eventually becomesvertical, leading to further stretching of plantarligaments, namely calcaneonavicular ligament, whichleads to the characteristic rocker-bottom foot. Thus,the plantar aspect of the foot is convex, and the dorsalaspect has a deep crease. The equinus position of thecalcaneus can further lead to subluxation ordislocation of the calcaneocuboid joint. Within theankle, only the posterior one third of the talar domearticulates as the calcaneus is plantarflexed androtated posterolateral. Sustenticulum tali is hypoplasticand provides no support to talar head. The posteriortibial tendon and both the peronei are subluxatedanterior to their respective malleoli, which thenfunction as ankle dorsiflexors rather than plantarflexors.[10]

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PHYSICAL EXAMINATIONAND CLINICAL FEATURES

Paediatric flat foot is one of the most commonconditions seen in paediatric orthopaedic practice. Theclinician should distinguish between a rigid andnon-rigid deformity. The differential diagnosis caninclude positional calcaneovalgus deformity,posteromedial bowing of tibia, congenital absence offibula, congenital oblique talus, idiopathic flat feet orCVT.[11]

CVT patients usually present with “Persian slipperfoot―,[9] a hindfoot equinus ,hindfoot valgus, forefootabduction and forefoot dorsiflexion, which are featurespresent in all new born with a vertical talus. The rigidityof these deformities is the key to diagnosis. Also, thetalar head is usually palpable at the medially at thesole of the foot. Since this condition is frequentlyassociated with other neuromuscular and geneticconditions, thus a comprehensive examination of thepatient should be performed. Any features such asfacial dysmorphic features, or spinal dyraphism shouldalert the clinician to get a genetic or neurologicalworkup. Sacral dimple, in particular should alert theexaminer about a central nervous system anomalies.

Documenting the motor function of the extensors andflexors of the toes also plays an important role.Stimulating the dorsal and plantar aspect of foot, in aserial manner, leads to dorsiflexion or plantarflexion

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response of the toes, This response is gradedseparately for the greater toe and for the other fourtoes and rated as absent, slight or definitive. Anabsent or slight response is indicative of a more rigidCVT. These responses may also be indicative of aneurological or muscular anomaly.[12]

Clinically, the plantar surface is convex with aprominent talar head, the dorsal surface presents withdeep creases and on palpation a gap can be feltwhere the normal talonavicular joint would havearticulated. If this gap reduces with plantarflexion, thenthe deformity can be considered to be flexible. Thispredicts responsiveness to treatment.

CVT does not delay weight bearing and these patientshave a peg-leg gait ( an awkward gait with limitedforefoot push-off)[9] with callus under a prominent talarhead. The heel does not touch the ground; ratherweight bearing occurs only in a half-dollar sized area.With time, shoe wear becomes difficult and painfulcallosities develop, making ambulation a painstakingjob.[13][14]

RADIOGRAPHICEVALUATION

Radiographic evaluation of paediatric foot is difficultdue to the non-ossified nature of the midfoot bones innew borne. Hindfoot and metatarsals are ossified atbirth, with midfoot bones appearing subsequently.Cuboid ossifies during the first month and cuneiformand navicular during second and third yearrespectively. An AP view and three lateral views inmaximal dorsiflexion, maximal plantarflexion andneutral should be done. The plantarflexion lateral viewis important to assess the rigidity of the talonaviculardislocation. The assessment should primarily focus onrelationship between the ossified centres rather thanthe midfoot bones, which can misguide theclinician.[15][16]

Useful angles on the lateral view include thetalocalcaneal angle, tibiocalcaneal angle and thetalar-first metatarsal axis angle. On a neutral view, thelong axis of talus is vertical in relation to firstmetatarsal; calcanuem is in significant equinus asseen with the high tibiocalcaneal angle. The forceddorsiflexion and plantarflexion views are important tohelp assess the rigidity of the deformity and thusdifferentiate CVT from more flexible deformities likeoblique talus or calcaneovalgus foot. In a case of CVT,a lateral view in plantarflexion demonstrates persistentvertical orientation of talus and a view in dorsiflexiondemonstrates persistent rigid hindfoot equinus. On the

lateral view in plantarflexion, the talar-first metatarsalaxis angle can be used to distinguish vertical andoblique talus, with angles >35degrees considered asdiagnostic for CVT. However an angle < 35 degreescannot rule out CVT. Next step in such cases shouldbe looking for the hindfoot equinus deformity. If rigid,then such feet need treatment in a manner similar toCVT feet. An AP talocalcaneal and talar-firstmetatarsal axis angle can be used as guides, but noangle is pathognomic of this deformity in AP views.[12]Of other imaging modalities, like MRI provide adetailed outline of the cartilage anlage of bones,though use is presently limited to research purposesonly.[9]

MANAGEMENT

Restoration of normal alignment of bones of foot andweight bearing capacity of first ray are primarily aimsof treatment. Depending on severity and rigidity ofdeformities multiple extensive as well as minimalinvasive surgical procedures have been described forCVT.

Closed treatment and casting, was one of the firsttreatment strategies used and showed poor resultsconsistently when done as a sole method ofmanagement. Jacobsen and Crawford’sperformed a literature review in 1983, whichemphasized that surgically managed patients hadbetter outcomes as compared to the conservativegroup.[5] Presently, the most often used protocol is ofserial casting which helps to stretch the foot in plantarflexion and inversion while counterpressure is appliedto the medial aspect of talus, followed by surgery forreduction of talo-navicular joint.[4] This serial castingand stretching also avoids compression of thenavicular head into talus.Any surgical treatment ispreferably delayed till the child is 12-18 months old.Earlier surgeries, especially those done before 2 yearsof age, tend to have a better prognosis.[12] Lamy andWeissman[17] in 1939 recommended excision of talusfor rigid flatfoot where as Eyre-Brooke[2] advocatedexcising the navicular. At present, neither of thesetechniques is accepted.

 Osmond-Clarke (1956),[3] Herndon andHeyman(1963)[16] and Coleman and colleagues[18](1970) described a staged, two-incision surgicaltechnique. Stage 1 included lengthening of EDB, EHL,Tibialis anterior and capsulotomies of talonavicularand calcanecuboid joints. Second stage includedTendo-achilles lengthening with a posteriorcapsulotomy of ankle and subtalar joints.

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In 1979, Ogata and colleagues[19] recommended asingle-stage approach noticing the high complicationrate following the staged procedures. In 1987, Seimoncame forward wi th a s ingle stage dorsalapproach.[1]Seimon propagated the idea of correctingboth the forefoot and hindfoot deformities. In hisapproach, EHL and Peroneous Tertius weretenotomised and talonavicular joint was opened. Thisjoint was further f ixed with a K-wire and apercutaneous TA tenotomy was done. This procedureyielded very good results in their study, leading themto conclude that there was no need of extensivesurgery or a two-staged surgery to be done. Stricker etal[20]published their experience with the Seimon’sone-stage method, and found very good results toobtain a plantigrade feet at 41 months despite thepresence of ankle and subtalar stiffness and someevidence of residual forefoot abduction on radiographs.

Mazzocca et al[21] in their study, compared the resultsbetween Seimon’s one stage surgery andposterior approach for CVT. They found similarradiographic results with both the procedures, but theyfound that posterior approach had more complications,increased operative time and poorer clinical scores.

Kodros et al[22] described a single-stage approachwith Cincinnati incision, an incision from medialcuneiform to lateral malleolus that could be extendedto calcaneocuboid joint. They reported good clinicaland radiological results, with normalization of AP andlateral talo-calcaneal and talar-first metatarsal angles,but a number of feet in their series neededre-surgeries.

Dobbs et al[4] presented their method of treatment ofCVT, via even less surgery as compared to previousguidelines. They combined closed manipulation,K-wire fixation of talonavicular joint and percutaneousheel cord release. They noticed that earlier proceduresof extensive surgical release were associated withcomplications like postoperative stiffness, woundnecrosis, AVN of talus, pseudo-arthrosis,under-correction of deformities as well as need forfurther surgeries.

Casting was done a method similar to Ponseti’smethod for clubfoot, with forces applied in oppositedirection and medial talar head used as fulcrum forcorrect ion. The foot is manipulated into aplantarflexion and inversion position with one handand the thumb of the other hand is used to push thetalar head into a dorsal and lateral position. Calcaneusis not manipulated at this stage. All deformities of CVTare corrected in a sequential manner, except equinuswhich is corrected last (Reverse Ponseti Technique).After a couple of minutes of stretch, a long leg plaster

is applied in a fashion similar to the clubfoot casts, butwith foot in equinovarus. Cast application should startas soon as possible and usually takes about 5 to 6casts for complete stretching of soft tissue and abilityto reduce talonavicular joint. The position in the finalcast is of extreme equinovarus resembling a clubfoot.This is followed by fixation of a reduced talo-navicularjoint. For this a dorsomedial incision is given andinspection of congruent joint reduction is done. If thejoint is reduced a retrograde K-wire is passed withoutopening the capsule. If the joint is not congruentlyreduced, capsulotomy is done and K-wire is passedafter reduction. K wire is buried to avoid backing out.In non-isolated, rigid CVT there is also contracture ofother soft tissue structures like tibialis anterior,peroneous brevis or dorsal extensor tendons whichare then lengthened. Finally, a percutaneous TendoAchilles tenotomy is done to correct the residualequinus. The foot is immobilized in cast with forefootand hindfoot in neutral and changed at 2 weeks to add10 degrees dorsiflexion. K-wire is removed at 6 weekspostoperatively followed by a shoe-bar-brace systemworn fulltime for 2 months, followed by night timewearing for 2 years, to prevent relapse. It is imperativeto ensure that there is no dorsiflexion in the bootsused with the bar. The parents are also taughtfrequent stretching of the feet, especially at diaperchange to reduce the chances of relapse. Once thechild starts walking he is given a day-time brace andan Ankle-Foot Orthosis to walk. Since the year 2006,multiple studies have been published which validatethis method of management as uniformly producinggood results.[10][23][24][25]

SUMMARY

CVT is an uncommon foot deformity and a cause forrigid flat or convex (rocker-bottom) feet in children. Anon-suspecting clinician can easily miss the diagnosisin view of looking only for clubfoot in new born andthus lead to a prolonged and tedious path ofmanagement. A thorough clinical examination,suspicion and radiology can allow the orthopedician todiagnose as well as start management as soon aspossible thus improving outcomes. Management hasdrastically changed from earlier methods of extensivesoft-tissue release procedures to the present favouriteof manipulation, casting and minimal invasive surgery.This method has yet produced uniformly good results,although long term results are still to be looked for.

BIBLIOGRAPHY

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1.Seimon LP. Surgical correction of congenital verticaltalus under the age of 2 years. J Pediatr Orthop.7(4):405-11.

2.Eyre-Brook AL. Congenital vertical talus. J BoneJoint Surg Br. 1967 Nov;49(4):618–27.

3.OSMOND-CLARKE H. Congenital vertical talus. JBone Joint Surg Br. 1956 Feb;38-B(1):334-41.

4.Dobbs MB, Purcell DB, Nunley R, Morcuende JA.Early Results of a New Method of Treatment forIdiopathic Congenital Vertical Talus. J Bone Jt Surg.2006 Jun;88(6):1192-200.

5.Jacobsen ST, Crawford AH. Congenital vertical talus.J Pediatr Orthop. 1983 Jul;3(3):306-10.

6.Sharrard WJ, Grosfield I. The management ofde fo rm i t y and pa ra l ys i s o f t he foo t i nmyelomeningocele. J Bone Joint Surg Br. 1968Aug;50(3):456-65.

7.Merrill LJ, Gurnett CA, Connolly AM, Pestronk A,Dobbs MB. Skeletal Muscle Abnormalities and GeneticFactors Related to Vertical Talus. Clin Orthop RelatRes. 2011 Apr 20;469(4):1167-74.

8.Dobbs MB, Gurnett CA, Pierce B, Exner GU,Robarge J, Morcuende JA, et al. HOXD10 M319Kmutation in a family with isolated congenital verticaltalus. J Orthop Res. 2006 Mar;24(3):448-53.

9.Drennan JC. Congenital vertical talus. Instr CourseLect. 1996;45:315-22.

10.Alaee F, Boehm S, Dobbs MB. A new approach tothe treatment of congenital vertical talus. J ChildOrthop. 2007 Sep;1(3):165-74.

11.Sankar WN, Weiss J, Skaggs DL. Orthopaedicconditions in the newborn. J Am Acad Orthop Surg.2009 Feb;17(2):112-22.

12.Miller M, Dobbs MB. Congenital Vertical Talus. JAm Acad Orthop Surg. 2015 Oct;23(10):604-11.

13. HARRIS RI, BEATH T. Hypermobile flat-foot withshort tendo achillis. J Bone Joint Surg Am. 1948Jan;30A(1):116-40.

14.Brand RA. 50 years ago in CORR: Congenitalvertical talus. Tom Outland MD and Henry H. SherkMD CORR 1960;16:214-218. Clin Orthop Relat Res.2010 Oct;468(10):2822-4.

15.Vanderwilde R, Staheli LT, Chew DE, Malagon V.Measurements on radiographs of the foot in normalinfants and children. J Bone Joint Surg Am. 1988Mar;70(3):407-15.

16.Mckie J, Radomisli T. Congenital Vertical Talus: ARev iew . C l i n Pod ia t r Med Su rg . 2010

Jan;27(1):145-56.

17.D Duncan RD, Ed F, Fixsen JA. THE JOURNALOF BONE AND JOINT SURGERY Congenital convexpes valgus. J Bone Joint Surg [Br]. 1999.

18.Co leman SS, S te l l ing FH, Jar re t t J .Pathomechanics and treatment of congenital verticaltalus. Clin Orthop Relat Res. 70:62-72.

19.Ogata K, Schoenecker PL, Sheridan J. Congenitalvertical talus and its familial occurrence: an analysis of36 patients. Clin Orthop Relat Res. (139):128-32.

20.Stricker SJ, Rosen E. Early One-StageReconstruction of Congenital Vertical Talus. FootAnkle Int. 1997 Sep 28;18(9):535-43.

21.Mazzocca AD, Thomson JD, Deluca PA, RomnessMJ. Comparison of the posterior approach versus thedorsal approach in the treatment of congenital verticaltalus. J Pediatr Orthop. 21(2):212-7.

22.Kodros SA, Dias LS. Single-stage surgicalcorrection of congenital vertical talus. J Pediatr Orthop.19(1):42-8.

23.Chalayon O, Adams A, Dobbs MB. MinimallyInvasive Approach for the Treatment of Non-IsolatedCongenital Vertical Talus. J Bone Jt Surgery-AmericanVol. 2012 Jun 6;94(11):e73-7.

24.Rodriguez N, Choung DJ, Dobbs MB. RigidPediatric Pes Planovalgus: Conservative and SurgicalTreatment Options. Clin Podiatr Med Surg. 2010Jan;27(1):79-92.

25.Eberhardt O, Fernandez F, Wirth T. DieBehandlung des Talus verticalis mit der Methode nachDobbs. Z Orthop Unfall. 2011 Apr 5;149(02):219-24.Â

AUTHORS CONTRIBUTION

Dr. Karthick S. R

Concept, Acquisition of data, Revision of Article

Dr. Pulak Vatsya

Drafting of Article, Concept and Design

Dr. Nirmal Raj Gopinathan

Critical Revision of Article

Dr. Prashant Sharma

Concept and Acquisition of data.

Illustrations

Illustration 1: Clinical picture of child showing

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rocker- bottom foot.

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Illustration 2: Lateral view with forced plantar flexionshowing vertical talus (talar- first metatarsal axis angle> 35?) on left foot.

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Illusration 3: AP radiograph of foot showing Angle X– Talar first metatarsal axis angle and Angle Y –Talo calcaneal angle.

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Illustration 4: Post op Xray showing Talo navicular jointreduced and fixed with a K wire.

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