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:
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
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4. Raghav Saini, Shivinder Singh Gill, Mandeep Singh
Dhillon, Results of dorsal approach in surgical
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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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9. John G. Thometz, MD, PhD, Hongsheng Zhu, MD, PhD,
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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
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