BY:DR.GIRISH MOTWANI

ORTHOPAEDICSB.J WADIA HOSPITAL FOR CHILDRENS.

CONGENITAL VERTICAL TALUS

CVT- Rare defomity

Term-1st used by:Henken in 1914.

Several Synonyms-

Congenital convex pes valgus(CCPV) Reverse club footcongenital valgus flatfootRocker buttom foot Talipes convex pes valgus

“teratologic dorsolateral dislocation of the talocalcaneonavicular joint.”

resulting in a rigid flatfoot deformity.

Incidence 1 in 10,000

Male=female

B/L -50%

Tachdjian M: Pediatric Orthopedics, vol 4. 2nd ed. Philadelphia, WB Saunders, 1990. Jacob sen ST,Crawford AH(1983)Congenital vertical talus. J Pediatr Orthop 3:306–310

Etiology

The exact etiology of vertical talus in most cases is not known.

Theories include increased intrauterine pressure and resultant tendon contractures,

or an arrest in fetal develop- ment occurring between the 7th and 12th week of gestation

50% idiopathic. Approximately one-half of all cases of

vertical talus occur in association with neurologic abnormalities or genetic syndromes

A/W -Neurological abnormalities-arthrogryposis,myelomeningocoele,spinal muscular atrophy,neurofibromatosis,cerebral palsy

-Genetic syndrome:trisomy 13,15 and 18

A thorough neurological and genetic work up

AD inheritance 12-20%

Mutation in HOXD10

Mutation in GDF5Syndromes-1.De barsy syndrome 2.Prune Belly syndrome 3.Costello syndrome 4.Rasmussen syndrome

Ogata and schoenecker –Three group-1-Idiopathic2-A/W other abnormality but no neurological

defecit3.A/W neurological defecit

Clinical Orthopaedics (1979 )139:128–132

3.Hamanishi:five groups- 1.NTD or spinal anomalies 2.neuromuscular disorders 3.malformation syndromes 4.chromosomal aberrations 5.idiopathic

J Pediatric Orthopaedics (1984)4:318–326

Irreducible dorsal & lateral dislocation of navicular over talus

Posteriorly, Contracture of tendoachillis creates equinus of calcaneus

Anteriorly,contracture of EDL(EHL,TIB ANT)

Laterally PL,PB ,calcaneofibular ligament contracted

Posterior tendons subluxation over malleolus

Pathoanatomy:

Navicular – hypoplastic wedge shaped

Talar head- flattened, extreme planter flexion,medially deviated

Calacaneum-plantar flexion ,ext rotated

Angle between axis of talus & calcaneum is increased

Cuboid in very severe case

pathoanatomy

Coleman classification

Coleman divided CVT into 2 types: type 1 was associated with a calcaneocuboid

dislocation, and type 2 was not. This distinction is important clinically

because the type 1 deformity is stiffer and particular attention must be paid to releasing the calcaneocuboid joint

Clinical presentation-

Forefoot-abduction ;dorsiflexion

Hindfoot-equinus and valgus

Plantar surface is convex-Rocker bottom appearance

Deep creases on anterolateral aspect of foot

Foot is everted into valgus and externally rotated position

Head of talus plantar medial aspect of midfoot

Calcaneus is in equinus

The forefoot is dorsiflexed at the midtarsal joints creating a palpable gap dorsally between the navicular and where the talar neck should normally be located. This gap can be helpful in distinguishing congenital vertical talus from the more common calcaneovalgus foot

What happen if untreated?

Heel doesnot touches the ground, have poor push off

Wt bearing on talar head resulting in painful callosities

Ambulation is usually not delayed but gait is awkward with difficult in balancing

Forefoot become severly abductedTalus become like “hourglass”Abnormal shape of foot result in difficult

shoewearing.

Radiological evaluation.

The lack of ossification of many of the bones in the foot at birth can make the diagnosis of congenital vertical talus challenging on plain radiographs

The talus, tibia, calcaneus, and metatarsals are ossified at birth.

The cuboid ossifies in the first month of life while the cuneiforms and navicular usually ossify around the ages of 2 and 3 years, respectively.

Since most children with vertical talus are seen in the newborn period, the radio- graphic evaluation is focused on the relationships of the ossified talus and calcaneus to the tibia as well as the relationship of the metatarsals to the hindfoot.

Forced plantar flexion and forced dorsiflexion lateral radiographs are necessary to confirm the diagnosis of vertical talus and rule out the oblique talus and calcaneovalgus foot as diagnoses. PLANTARFLEXED FILM:The forced plantar flexion lateral radiograph in a vertical talus foot shows persistent malalignment of the long axis of the talus and the first metatarsal.it show persistent dorsal translation of the forefoot on the hindfoot. DORSIFLEXED FILM: the forced dorsiflexion lateral radiograph demonstrates a persistently decreased tibiocalcaneal angle indicating fixed hindfoot equinus .OBLIQUE TALUS:In contrast, a forced plantar flexion lateral radiograph of an oblique talus will demonstrate restoration of a normal relationship between the long axis of the talus and the first metatarsal

Measurements that can be obtained on the lateral radiograph include

the talocalcaneal, tibiocalcaneal, tibiotalar, and talar axis- first metatarsal base angles.

Hamanishi described 2 radiographic angles: the talar axis–first metatarsal base angle

(TAMBA) and the calcaneal axis–first metatarsal base

angle (CAMBA).

Congenital vertical talus: classification with 69 cases and new measurement system. Hamanishi C. Abstract Sixty-nine cases of congenital vertical talus (CVT) were classified into five groups in

association with (1) neural tube defects or spinal anomalies, (2) neuromuscular disorders, (3) malformation syndromes, (4) chromosomal aberrations, and (5) idiopathic CVT unassociated with any of the systemic conditions described above. Forty-four cases of idiopathic CVT were subclassified into four groups: (5A) intrauterine molded or deformed cases, (5B) cases of digitotalar dysmorphism associated with contractile finger abnormalities and genetic inheritance, (5C) patients whose close relatives had CVT or oblique talus (OT) deformity, and (5D) cases unassociated with any skeletal deformity or genetic inheritance.

“The talar and Calcaneal axis--first metatarsal base angles (TAMBA and CAMBA) are introduced, which enable us to describe not only the obliquity of the talus and calcaneus but also the severity of the dislocation of the talonavicular joint and the contracture of the tendo Achilli.”

The changing point from flexible OT to rigid CVT is TAMBA of about 60 degrees and CAMBA of 20 degrees, and there are many borderline cases of CVT that could be treated conservatively. For the typical CVT, open reduction should be carried out as promptly as possible if 3 months of corrective casting in extreme equinovarus fails to reduce the TAMBA to 50 degrees.

Hamanishi C. Congenital vertical talus: classification with 69 cases and new measurement system. J Pediatr Orthop. 1984 May. 4(3):318-26

Role of USG

Pediatr Radiol. 2013 Mar;43(3):376-80. doi: 10.1007/s00247-012-2529-5. Epub 2012 Nov 27.

Dynamic US study in the evaluation of infants with vertical or oblique talus deformities.

Supakul N1, Loder RT, Karmazyn 

 radiographs of an infant's foot particularly less than 6 months can be difficult to interpret. The use of dynamic ultrasound has been reported to be helpful in the evaluation of infants with vertical or oblique talus.

Differentials-

Calcaneovalgus foot deformity: -foot is dorsiflexed -no equinus contracture of

calcaneus -flexible foot -forced plantar flexion lateral x-ray-

normalPosteromedial bow of the

tibia:calcaneovalgus foot,a shortened and bowed tibia

Oblique talus

Treatment .

The goals of treatment are to restore the normal anatomic relationships between the talus, the navicular, and the calcaneus, in order to provide a normal weight distribution through the foot.

There are multiple surgeries described for the treatment of vertical talus.

The type of procedure used for an individual patient is based on

the age of the patient, severity of the deformity, and the preference of the surgeon.

Children up to the age of 3 years are usually offered an open reduction of the talonavicular joint, which can be performed through either a one-stage or two-stage operation

Traditional procedures.

Several authors, beginning with Osmond-Clarke, Herndon and Heyman, and Coleman and associates, described staged, 2-incision reconstructive surgery.

The first stage of the Coleman procedure consisted of lengthening the extensor digitorum longus (EDL), extensor hallucis longus (EHL), and tibialis anterior, with capsulotomies of the talonavicular and calcaneocuboid joints and release of the talocalcaneal interosseous ligament.

The second stage consisted of tendo-Achilles lengthening (TAL) and a posterior capsulotomy of the ankle and subtalar joints.

Coleman SS, Stelling FH 3rd, Jarrett J. Pathomechanics and treatment of congenital vertical talus. Clin Orthop Relat Res. 1970 May-Jun. 70:62-72.

Herndon CH, Heyman CH. Problems in the recognition and treatment of congenital pes valgus. J Bone Joint Surg Am. 1963. 45:413-29.

Osmond-Clarke H. Congenital vertical talus. J Bone Joint Surg Br. 1956 Feb. 38-B(1):334-41. 

Then trend changed to single stage technique.

After noting a high incidence of complications with the 2-stage technique, Ogata and colleagues recommended a single-stage procedure with a medial approach

Kodros and Dias published results they derived using a single-stage approach with a Cincinnati incision.

Seimon described a single-stage dorsal approach

Three basic components

The first step is the reduction of the talonavicular joint which is aided by release of the anterior tibialis tendon and the tibionavicular and talonavicular ligaments. The reduction is held by a Kirschner wire placed across the talonavicular joint

. The second step is lengthening of the toe extensors and pero- neals which aids in improving ankle plantar flexion and forefoot adduction. The calcaneocuboid joint is also reduced if necessary.

The third step is correction of the ankle equinus contracture which is done by lengthening the Achilles tendon and releasing the ankle and subtalar joint capsules

. Some authors have recommended the addition of a tibialis anterior tendon transfer to the head or neck of the talus at the time of open reduction to add a dynamic corrective force

Modified cincinnati incision-

The Cincinnati incision provided excellent exposure to the pathoanatomy to allow complete correction of the plantarflexed vertical talus, reduction of the talonavicular dislocation, and realignment of the equinovalgus deformity of the calcaneus.

Kodros, Steven A. M.D.*; Dias, Luciano S. M.D. Single-Stage Surgical Correction of

Congenital Vertical Talus. Journal of Pediatric Orthopaedics; 19(1), January/February 1999, pp 42-48

Single stage repair-

Three incisions-

Through DL approach-calcaneocuboid joint inspected and reduced

Medially,dorsal talonavicular ligament (deltoid)divided and capsulotomy of talonavicular joint done; reduced and transfixed with k-wire.

Postriorly,Z-lengthening of Achilles tendon with distal transverse cut directed laterally.

Check lateral x-ray:1st metatarsal axis should line up exactly

with long axis of talus

COMPLICATIONS.

Correction of vertical talus through an open reduction can be associated with significant short-term complications, including

wound necrosis undercorrection of the deformity , stiffness of the ankle and subtalar joint , and the eventual need for multiple operative

procedures such as subtalar and triple arthrodesis . Long-term outcomes are likely to be complicated by a

significant amount of degenerative arthritis as is seen in many patients with clubfoot treated with extensive soft-tissue releases

Matthew B Dobbs, MD

Recognized for his skill at treating all paediatric foot disorders.

Minimally invasive approach toward the treatment of CVT.

Between 2000 to 2003, at St. Louis Children’s Hospital & University of Iowa Hospitals and Clinics ;Dobbs et al treated 11 cases (19 feet) of idiopathic CVT by:

-serial manipulation and casting(reverse ponseti technique),

-percutaneous fixation of talonavicular joint using k- wire and

- percutaneous Achilles tenotomy.

REVERSE PONSETI CASTING

The foot is stretched into plantar flexion and inversion while counter pressure is applied to the medial aspect of the head of the talus

4-6 plaster cast is usually enough to achieve reduction of the talonavicular joint

Final cast –Maximum plantar flexion,inversion

Foot simulates –clubfoot Lateral radigraph in PF;TAMBA<30’

Dobbs minimally invasive technique-

After the talonavicular joint has been reduced(after 5-6 casts),fixed percutaneously with k-wire.

Wire passed retrogade from the navicular into the talus with foot in maximum plantiflexion

Wire bent and cut outside skin

Dobbs minimally invasive technique

Even after 6 cast talonavicular joint is not seen to be reduced (TAMBA>30) then an attempt is made in the operating room to lever the talus into position percutaneously with a k-wire placed into the talus in a retrograde manner.

If this is successful, the talonavicular joint is held with k-wire.

Dobbs minimally invasive technique

If the talonavicular joint not reduced closed,a small medial incision is made and dorsal capsulectomy of talonavicular joint was done to reduce the joint.

Fractional lengthening of tibialis anterior and peroneus brevis tendon.

Once talonavicular joint reduced and fixed with k-wire

percutaneous tenotomy was done.

AFTER TA TENOTOMY …….

An assessment is made of the ankle plantar flexion and forefoot passive adduction at this point. If plantar flexion is limited to <25, a fractional lengthening of the extensor digitorum communis is done at the level of the musculotendinous junction.

If passive forefoot adduc- tion is <10, fractional lengthening of the peroneal brevis tendon is performed at the musculotendinous junction.

Lengthening of the peroneal brevis and extensor digitorum communis is not often needed since the preoperative casting usually stretches these structures enough

Dobbs Post op protocol

After tenotomy,a long leg cast :foot –neutral Ankle 5’ DFCast changed at 2 weeks (Mold is made for

solid AFO with 15’ of PF at midtarsal joint)A long leg cast –ankle in 10-15’DF x 3

weeks

After 5 wks;cast removed and k-wire pulled

The solid orthoses is applied and parents are instructed regarding exercise and ankle ROM.

Orthoses is worn for 23 hrs a day until walking age.

Then 12-14 hrs a day until the age of 2 years.

After bracing every 3 monthly until age of 2 yrs

Then every 6 month-1 yr until age of 7 yrsAfter 7,once every 2 yr until skeletal maturity is

reached

Routine follow up assessment

Both clinical and radiological parameter.Clinical-1.ankle and subtalar movement 2.cosmetic appearance 3.loss of the medial arch 4.medial prominence of the talar

head 5.hind foot valgus 6 .abnormal shoe wear

Radiological –anteroposterior: 1.talocalcaneal –hindfoot algus 2.TAMBA-forefoot abduction lateral: 1.talocalcaneal 2.tibiocalcaneal 3.TAMBA

Outcome measures

As by Adellar et al-Comprises 10 point scale :6 clinical appearance 4 radiological

parameter Maximum 10 points –Excellent 7-9 -good 4-6 -fair <3 -poor

Bone Joint J 2014;96-B:274–8

Excellent results, in terms of the clinical appearance of the foot, foot function, and deformity correction as measured radiographically , in patients with idiopathic and those associated with other genetic or neuromuscular disorder ;congenital vertical talus.

J Child Orthop 2007;1:165–174 J Bone Joint Surg [Am] 2012;94-

A:73. J Bone Joint Surg [Am] 2006;88- A:1192–1200.

J Bone Joint Surg Am. 2006 Jun;88(6):1192-200. Early results of a new method of treatment for idiopathic congenital vertical talus. Dobbs MB1, Purcell DB, Nunley R, Morcuende JA.

Abstract BACKGROUND: The treatment of idiopathic congenital vertical talus has traditionally consisted of manipulation and application of casts followed by extensive

soft-tissue releases. However, this treatment is often followed by severe stiffness of the foot and other complications. The purpose of this study was to evaluate a new method of manipulation and cast immobilization, based on principles used by Ponseti for the treatment of clubfoot deformity, followed by pinning of the talonavicular joint and percutaneous tenotomy of the Achilles tendon in patients with idiopathic congenital vertical talus.

METHODS: The cases of eleven consecutive patients who had a total of nineteen feet with an idiopathic congenital vertical talus deformity were

retrospectively reviewed at a minimum of two years following treatment with serial manipulations and casts followed by limited surgery consisting of percutaneous Achilles tenotomy (all nineteen feet), fractional lengthening of the anterior tibial tendon (two) or the peroneal brevis tendon (one), and percutaneous pin fixation of the talonavicular joint (twelve). The principles of manipulation and application of the plaster casts were similar to those used by Ponseti to correct a clubfoot deformity, but the forces were applied in the opposite direction. Patients were evaluated clinically and radiographically at the time of presentation, immediately postoperatively, and at the time of the latest follow-up. Radiographic measurements obtained at these times were compared. In addition, the radiographic data at the final evaluation were compared with normal values for an individual of the same age as the patient.

RESULTS: Initial correction was obtained both clinically and radiographically in all nineteen feet. A mean of five casts was required for correction. No

patient underwent extensive surgical releases. At the final evaluation, the mean ankle dorsiflexion was 25 degrees and the mean plantar flexion was 33 degrees . Dorsal subluxation of the navicular recurred in three patients, none of whom had had pin fixation of the talonavicular joint. At the time of the latest follow-up, there was a significant improvement (p < 0.0001) in all of the measured radiographic parameters compared with the pretreatment values, and all of the measured angles were within normal values for the patient's age.

CONCLUSIONS: Serial manipulation and cast immobilization followed by talonavicular pin fixation and percutaneous tenotomy of the Achilles tendon provides

excellent results, in terms of the clinical appearance of the foot, foot function, and deformity correction as measured radiographically at a minimum two years, in patients with idiopathic congenital vertical talus.

J Bone Joint Surg Am. 2012 Jun 6;94(11):e73. doi: 10.2106/JBJS.K.00164. Minimally invasive approach for the treatment of non-isolated

congenital vertical talus. Chalayon O1, Adams A, Dobbs MB. 1Department of Orthopaedic Surgery, Washington University School of Medicine, St. Louis, MO 63110, USA. Abstract BACKGROUND: Traditional extensive soft-tissue release for the treatment of congenital vertical talus is associated with a myriad of

complications. A minimally invasive approach has recently been introduced with good short-term results in patients with isolated vertical talus. The purpose of the present study was to evaluate the effectiveness of this approach for the treatment of rigid vertical talus associated with neuromuscular and/or genetic syndromes.

METHODS: Fifteen consecutive patients (twenty-five feet) with non-isolated congenital vertical talus were retrospectively

reviewed at a minimum of two years following treatment with serial casting followed by limited surgery. The surgery consisted of percutaneous Achilles tenotomy in all feet and either pin fixation of the talonavicular joint through a small medial incision to ensure joint reduction and accurate pin placement (five feet) or selective capsulotomies of the talonavicular joint and the anterior aspect of the subtalar joint (twenty feet). Patients were evaluated clinically and radiographically at the time of presentation, immediately postoperatively, and at the time of the latest follow-up. Radiographic data at the time of the latest follow-up were compared with age-matched normative values.

RESULTS: Initial correction was obtained in all cases. The mean number of casts required was five. Mean ankle dorsiflexion was 22° and

mean plantar flexion was 25° at the time of the latest follow-up. Recurrence was noted in three patients (five feet), all of whom had had initial subluxation of the calcaneocuboid joint. All radiographic parameters measured at the time of the latest follow-up had improved significantly (p < 0.0001) compared with the values before treatment, and the mean values of the measured angles did not differ significantly from age-matched normal values.

CONCLUSIONS: Serial manipulation and casting followed by limited surgery, consisting of percutaneous tenotomy of the

Achilles tendon and a small medial incision to either palpate the talonavicular joint or perform capsulotomies of the talonavicular joint and the anterior aspect of the subtalar joint to ensure accurate reduction and pin fixation, result in excellent short-term correction of the deformity while preserving subtalar and ankle motion in patients with rigid congenital vertical talus associated with neuromuscular and/or genetic syndromes

Indian J Orthop. 2008 Jul-Sep; 42(3): 347–350. doi:  10.4103/0019-5413.41860 PMCID: PMC2739479 Congenital vertical talus: Treatment by reverse ponseti technique Atul Bhaskar.

Abstract Background: The surgery for idiopathic congenital vertical talus (CVT) can lead to stiffness, wound complications and under or over

correction. There are sporadic literature on costing with mixed results. We describe our early experience of reverse ponseti technique.

Materials and methods: Four cases (four feet) of idiopathic congenital vertical talus (CVT) which presented one month

after birth were treated by serial manipulation and casting, tendoachilles tenotomy and percutaneous pinning of talonavicular joint. An average of 5.2 (range - four to six) plaster cast applications were required to correct the forefoot deformity. Once the talus and navicular were aligned based on the radiographic talus-first metatarsal axis, percutaneous fixation of the talo-navicular joint with a Kirschner wire, and percutaneous tendoachilles tenotomy under anesthesia was performed following which a cast was applied with the foot in slight dorsiflexion.

Results: The mean follow-up period for the four cases was 8.5 months (6-12 months). At the end of the treatment all feet were supple

and plantigrade but still using ankle foot orthosis (AFO). The mean talocalcaneal angle was 70 degrees before treatment and this reduced to 31 degrees after casting. The mean talar axis first metatasal base angle (TAMBA) angle was 60° before casting and this improved to 10.5°.

Conclusion:

Although our follow-up period is small, we would recommend early casting for idiopathic CVT along the same lines as the Ponseti technique for clubfoot except that the forces applied are in reverse direction. This early casting method can prevent extensive surgery in the future, however, a close vigil is required to detect any early relapse.

WHAT ABOUT OLDER CVT?

• Some children after the age of 3 years require excision of the navicular at the time of open reduction.

• Children between the ages of 4 and 8 years with either a primary or a recurrent deformity can be treated with open reduction combined with extraarticular arthrodesis (GRICE GREEN )

• Those patients that are older than 8 years often require a triple arthrodesis . However, arthrodesis does result in painful degenerative arthritis of the ankle and midtarsal joints when the patients are followed long-term