46 Kerala Journal of Orthopaedics Volume 26| Issue 1 | January 2013

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Congenital Vertical Talus in Freeman Sheldon Syndrometreated with Reverse Ponseti method

INTRODUCTION

The pathoanatomy of Congenital VerticalTalus has been well documented. CongenitalVertical Talus was first described by Henken in 1914,characteristic features was described by Lemi &

Weissmar in 1939. The exact etiology is stillunknown but over action of anterior tibial tendonin paralytic disorders and intrauterine compressionhas been described as a possible cause. Thedeformity may also occur following a degree ofgrowth arrest at 7th and 12th weeks of gestation.

HOX gene mutation may cause Congenital VerticalTalus in many families and in Charcot Marie toothdisease.

It is a rare deformity and initially surgery wasthe mainstay of deformity correction and the

treatment was according to age and severity ofdeformity. Surgical option included open reductionand realignment of talonavicular and subtalarjoints and a combination of several soft tissuereleases and subtalar and triple arthrodesis forpermanent correction of the deformity.

But over the last 4 years the possibility andefficacy of a new method of treatment forCongenital Vertical Talus has been explored. Themain components of the new methods are reversePonseti cast and a minimal invasive procedure.

Freeman-Sheldon syndrome (FSS) is a rare form

of the multiple congenital contracture

(arthrogryposis) syndromes, also known as

whistling face syndrome, characterised by

dysmorphic status combining bone anomalies andjoint contractures with typical facies features. FSS ispart of the nosologic group of the distalarthrogryposis. The three basic abnormalities aremicrostomia with pouting lips, camptodactyly ofthe hand and talipes equinovarus/ vertical talus.

CASE REPORT

A 8 months old male child presented withdeformity of both feet since birth. Antenatal historyrevealed that he was the first baby of diabeticmother born via LSCS and his birth weight 2.8Kg.

Examination revealed that fore foot was indorsiflexion and abduction whereas the hind footwas in valgus. The sole was convex with prominentmedial border and anterolateral and posterior crease.The degree of deformity was more in left foot

when compared to right foot. A head to footexamination revealed that child had Plagiocephaly,narrow palpebral fissure, high arched palate,Micrognathia, short neck and a low posterior hairline. Hands showed thumb in palm deformity andCamptodactyly of 2-5 fingers on both sides.

Considering the above mentioned featureswith CVT the diagnosis of Freeman Sheldonsyndrome was entertained. The confirmation ofdiagnosis of Congenital Vertical Talus was done

radiographically.

Mukesh Kumar1, Chandrababu KK2, Bhaskaran VK3

Amritha Institute of

Medical Sciences, Kochi,

Kerala, India

1 Resident in

Orthopaedics

2, 3 Consultanat in

Orthopaedics

Correspondence should

be sent to:

mukeshk@aims.amrita.edu

Kerala Journal of

Orthopaedics

2013;26(1):46-50

ABSTRACT

Authors report a case of Freeman-Sheldon syndrome with Congenital Vertical Talus in a 10 month

old child, which was treated by closed manipulation using a newer management protocol. Adeeliar Score

was used to assess the progress and outcome of treatment. The procedure involved serial manipulations

by Reverse Ponsetti method and casting followed by limited open redution and fixation.

The clinical and radiological results at the end of 18 months are good. The child is on an ankle foot

orthosis and kept under regular follow up.

The potential advantage of this method is that the structured serial manipulations stretch the soft

tissues. Hence the soft tissue release can be kept to a minimum, which also helps in open realignment of

the bones. Less soft tissue release also reduces scar tissue formation, thus maintaining the flexibility of

the foot.

Key words: Freeman-Sheldon syndrome, Congenital Vertical Talus, Reverse Ponsetti method

Case report

Kerala Journal of Orthopaedics Volume 26 | Issue 1 | January 2013 47

To proceed further we classified the Congenital Vertical

Talus According to Lichtblau’s & Coleman’s classification system.

On analysis, it was found that this case belonged to the group

Fig. 1a and b. Clinical photographs showing Rocker bottom foot with anterolateral crease an the dorsiflexed foot.

Fig. 2a. Clinical photograph showing posterior crease Fig. 2b. Clinical photograph showing Reverse Ponseti cast

: Lichtblau’s- Type 2 (Neurogenic), Coleman’s- Type 1

Table.1. Radiographic angles in

different view at presentation

TAMBA = Talar axis first metatarsal

base angle

Mukesh, Chandrababu and Bhaskaran.: Congenital Vertical Talus in Freeman Sheldon Syndrome treated with Reverse Ponseti method

Case report

ANGLES OBSERVED NORMAL

AP view R L

Talocalcaneal angle 40 43 20 - 40

TAMBA 28 43 -10 - 30

Lateral View

Talocalcaneal angle 58 65 35 - 50

Tibiocalcaneal angle 108 112 55 - 90

Tibiotalar angle 146 178 < 120

TAMBA 45 69 0 - 20

TAMBA (PF view) 38 61 0 - 20

48 Kerala Journal of Orthopaedics Volume 26 | Issue 1 | January 2013

We scored the deformity of foot according to Adeeliar

Score and it was 8/10. With the above preparations treatment

was started according to new method (Reverse Ponseti technique)

at 10 months of age. Serial manipulations and reverse Ponseti

casting was done and total of 9 casts was applied.

During the course of casting, deformity of feet was scored

after each casting and it was found that there was change in

clinical appearance of foot and improvement in Adeeliar score.

Following the 9th cast the situation was analysed both clinically

and radiographically.

Clinical assessment revealed a near normal appearance of

feet with anterior and lateral crease absent though posterior crease

persisted along with the heel valgus. Plantar flexion of 400 was

possible. Radiographic assessment of the included angles was

done as is shown in Table 2. Having met the prerequisite for

surgical correction, we proceeded to the surgical correction of

residual deformity, which was achieved by open reduction and K

wire fixation of TN joint after realigning and reducing the talus,

followed by percutaneous tenotomy of Tendoachilles tendon,

Five degree dorsiflexion groin to toe cast was given in immediate

postop period. After 3 weeks cast was removed and reapplied in

150 of dorsiflexion, after 5 weeks K wire was removed and brace

was given in 100 plantar flexion and 100 of adduction till walking

age. Ankle foot orthosis was given in with 150 plantar flexion

and 150 adduction. Throughout the follow up period parents

are counseled as to the importance of maintaining the achieved

result and being compliant. ROM exercises and foot inversion

were done 2-3 times per day.

At last follow up at 18 months of age, chiled is walking

with normal appearance of foot with normal range of motion.

of ankle. An AFO isbeing used for maintenance of correction

Fig. 3. X- ray AP view; TAMBA 310 Fig. 4. X- ray AP view; TAMBA 430

Fig. 5. X- ray Lat. view; Tibiotalar angle 1450 Fig. 6. X- ray Lat. view; Tibiotalar angle 1570

Mukesh, Chandrababu and Bhaskaran.: Congenital Vertical Talus in Freeman Sheldon Syndrome treated with Reverse Ponseti method

Case report

Kerala Journal of Orthopaedics Volume 26 | Issue 1 | January 2013 49

Table. 2.

Radiographic

angles before

and after casts

Fig. 7. Per op. photograph: isolation of Tib. Anterior Fig. 8. Per op. photograph: identifiation of Talonaicular joint

Fig. 8. Per op. photograph: Tendo Achilles tenotomyFig. 9. C-arm picture: K wire through Talus and Navucular

Mukesh et alMukesh, Chandrababu and Bhaskaran.: Congenital Vertical Talus in Freeman Sheldon Syndrome treated with Reverse Ponseti method

Case report

ANGLES OBSERVED (BC) OBSERVED (AC) NORMAL

AP view R L R L

Talocalcaneal angle 40 43 10 24 20 - 40

TAMBA 28 43 2 17 - 10 - 30

Lateral View

Talocalcaneal angle 58 65 49 51 35 - 50

Tibiocalcaneal angle 108 112 100 90 55 - 90

Tibiotalar angle 146 178 147 167 < 120

TAMBA 45 69 23 35 0 - 20

TAMBA (PF view) 38 61 1 19 0 - 20

50 Kerala Journal of Orthopaedics Volume 26 | Issue 1 | January 2013

Fig. 9. X - ray of the right foot at 18 months : Talus and Calcaneum remain reduced

Fig. 10. Photograph of the brace showing 150 dorsiflexion and 150 adduction at tarsometatarsal joint

REFERENCES

1. Bart H. Bosker, Jon H. M. Goosen, René M. Castelein,

Adriaan K. Mosert. Congenital convex pes valgus

(congenital vertical talus) The condition and its

treatment

2. Matthew B. Dobbs, MD, Derek B. Purcell, MD, Ryan

Nunley, MD, and Jose A. Morcuende, MD, PhD. Early

Results of a New Method of Treatment for Idiopathic

Congenital Vertical Talus.

3. R. D. D. Duncan, J. A. Fixsen, Congenital convex pes

valgus.

4. Raghav Saini, Shivinder Singh Gill, Mandeep Singh

Dhillon, Results of dorsal approach in surgical

correction of congenital vertical talus.

5. Atul Bhaskar, Congenital vertical Talus – treatment by

reverse ponseti technique. Indian J Orthop July-Sep;

42(3): 347-350

6. David A. Stevenson, MDa,b, John C. Carey, MDa,b, Janice

Palumbos, MSa, Ann Rutherford, Clinical

Characteristics and Natural History of Freeman-Sheldon

Syndrome.

7. Mark A. Katz, M.D., Richard S. Davidson, M.D., Peter S.

H. Chan, M.D., and R. J. Sullivan, Plain Radiographic

Evaluation of the Pediatric Foot and Its Deformities.

8. Jean Philippe Cahuzac, M.D.,* Jose´ Navascues, M.D.,*

Christiane Baunin, M.D.,w Je´roˆme Salles de Gauzy,

M.D. Assessment of the Position of the Navicular by

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Infant Foot Deformities.

9. John G. Thometz, MD, PhD, Hongsheng Zhu, MD, PhD,

Xue-Cheng Liu, MD, PhD, MRI Pathoanatomy Study of

Congenital Vertical Talus.

Mukesh, Chandrababu and Bhaskaran.: Congenital Vertical Talus in Freeman Sheldon Syndrome treated with Reverse Ponseti method

Cite this article as:Mukesh Kumar, Chandrababu KK, Bhaskaran VK. Congenital Vertical Talus in Freeman Sheldon Syndrome treated with Reverse Ponseti method.

Kerala Journal of Orthopaedics 2013;26(1):46-50

Source of funding: Nil; Conflict of interest: Nil

Case report