Congenital vertical talus

RELEVANT ANATOMY:

 Multiple pathoanatomical changes occur to varying degrees as a result of CVT.

 The navicular is displaced onto the dorsolateral aspect of the talar head and neck.
 This leads to the navicular becoming wedge-shaped with a hypoplastic plantar segment.
 The talar head is flattened dorsally, and its articular cartilage expands to accommodate
the articular surface of the displaced navicular.
 The sustenaculum tali is hypoplastic and provides no support to the talar head.
 The anterior and middle subtalar facets are either absent or replaced by fibrous tissue, and
the posterior facet is misshapen with increased lateral tilt.
 The cuboid is laterally displaced.
 The plantar half of the cuboid may be hypotrophic when a large degree of dorsal
subluxation or frank dislocation occurs through the entire transverse tarsal articulation.

DEFINITION: Uncommon disorder defined by rigid dorsal dislocation of the navicular on the
talar head and neck.

 Commonly associated with neuromuscular and genetic disorders, including trisomy

13, 14, 15, and 18.

 The navicular bone is dislocated dorsolaterally on the head

of the talus.

 Clinical features include a rigid convex plantar surface (rocker bottom) with hindfoot
equinus and hypoplastic laterally deviated forefoot.

EPIDEMIOLOGY:

 CVT has an incidence of 1 in 10,000 and affects males and females with equal frequency.
 Fifty percent of CVT cases present as an isolated (idiopathic) deformity, whereas the
other 50% occur in association with neuromuscular or genetic disorders.
 The cause of this deformity is unknown; however, existing evidence suggests that some
isolated deformities are transmitted as an autosomal dominant trait with incomplete
penetrance.

ETIOLOGY:
  CVT has been linked with defects of the central nervous system (CNS), muscle
abnormalities, acquired deformities, and certain genetic conditions.
 CNS defects associated with CVT include, but are not limited to, diastematomyelia,
lipoma of the cauda equina, myelomeningocele, sacral agenesis, arthrogryposis, and
neurofibromatosis
 An ischiocalcaneus band is a muscle abnormality also associated with CVT.
- It is a rare fibrous anlage of muscle that originates from the ischium, spans the
popliteal space, and blends distally into the aponeurosis of the triceps surae.
 This muscular abnormality is associated with a flexion contracture of the knee. Acquired
deformities associated with CVT include, but are not limited to, cerebral palsy, polio, and
spinal muscular atrophy.
 CVT can also be precipitated by overcorrection of a clubfoot deformity.

PATHOPHYSIOLOGY:

 The hallmark of CVT is an irreducible and rigid dorsal dislocation of the navicular on the
talus.
 Seimon hypothesized that a contracture of the tendo Achillis posteriorly creates equinus
of the calcaneus, with increased verticality of the talus, whereas contracture of the
EDL (and sometimes the EHL and the tibialis anterior) pulls the navicular onto the
dorsum of the navicular.

SIGNS AND SX:

- The most common symptom of congenital vertical talus is a rocker-bottom
appearance of the foot, which is usually obvious at birth or seen when a child begins
to walk.

Other symptoms include:

 An upward flex of the mid- and forefoot

 The hindfoot is elevated due to an abnormal flex in the ankle
 The midfoot cannot be properly aligned with the hindfoot
 Abnormal positioning of the foot; child may walk on the inside of their foot, while the
outside edge is elevated, leading to improper balance and weight distribution.

ASSESSMENT TOOL:

PODCI
  The Pediatric Outcomes Data Collection Instrument (PODCI) questionnaire is used to
quantify functional abilities of a group of unilateral upper extremity deficiency (U-UED)
patients and compare them with "normal" control children.
 The PODCI was developed specifically to assess changes following pediatric orthopedic
interventions for a broad range of diagnoses.
 These diagnoses include ambulatory children with cerebral palsy (CP), having Gross
Motor Function Classification System (GMFCS) levels of I to III, indicating motor
function that is minimally to moderately impaired.
 The PODCI items focus on function and quality of life of the child, attributes that might
change with surgical intervention. Reliance on changes in the PODCI to assess
effectiveness of intervention, however, requires an understanding of its items and
constructs.

Dx:

 The diagnosis is confirmed radiographically with anteroposterior and lateral weight

bearing or simulated weight-bearing views. A maximal plantar flexion lateral radiograph shows
the inability to align the forefoot and midfoot with the hindfoot.
 X-rays, which produce images of bones. The hallmark of this deformity is an abnormally
positioned talus bone (this is the bone that connects the foot to the ankle).
 EOS imaging, an imaging technology that creates 3-dimensional models from two flat
images. Unlike a CT scan, EOS images are taken while the child is in an upright or standing
position, enabling improved diagnosis due to weight-bearing positioning.

Prognosis:

 Minor calf atrophy and foot size asymmetry occur and are more noticeable in unilateral
cases.
 Ankle range of motion is about 75% of normal. If avascular necrosis (AVN) of the talus
occurs, the results are less optimal because of ankle pain, stiffness, and weakness.
 Several authors, beginning with Osmond-Clarke, Herndon and Heyman, and Coleman
and associates, described staged two-incision reconstructive surgery
 The first stage of the Coleman procedure consisted of lengthening the EDL, the EHL, and
the tibialis anterior, with capsulotomies of the talonavicular and calcaneocuboid joints
and release of the talocalcaneal interosseous ligament.
 The second stage consisted of TAL and a posterior capsulotomy of the ankle and subtalar
joints.
 Seimon described a single-stage dorsal approach in which the EHL and the peroneus
tertius were tenotomized and the talonavicular joint was opened.
  The talonavicular joint was reduced and held with a K-wire. The Achilles tendon was
lengthened percutaneously.
 Stricker and Rosen published their experience with this technique, as did Mazzocca et al;
both groups noted excellent results with few complications. 

MANAGEMENT

SURGICAL:

 Surgery is generally performed between 6 and 12 mo of age; a soft-tissue release is

performed as a 1 or 2 stage procedure.
 One component involves release/lengthening of the contracted anterior soft tissues in
concert with an open reduction of the talonavicular joint, while the other involves a
posterior release with lengthening of the contracted musculotendinous units.
 Fixation with Kirschner wires is commonly performed to maintain alignment.

REHAB:

 Extensive soft tissue releases of the midfoot and hindfoot joints combined with tendon
lengthening either in one or two stages have been described by many authors.
 Dobbs et al 7 published his technique on treatment of CVT which consists of serial
casting to align the forefoot to the hindfoot followed by talonavicular joint pinning and
Achilles tendon release.
 Occasionally, as part of the technique, anterior tibialis and/or peroneus brevis tendon
fractional lengthening may be required

REFERENCES:

https://orthoinfo.aaos.org/en/diseases--conditions/vertical-talus/

https://www.clinicalkey.com/#!/content/book/3-s2.0-B9781455775668006748?scrollTo=
%23hl0000622

https://www.clinicalkey.com/#!/content/journal/1-s2.0-S0263931916301521?scrollTo=
%23hl0000095

https://www.clinicalkey.com/service/content/pdf/watermarked/1-s2.0-
S0891842209000706.pdf?locale=en_US

https://emedicine.medscape.com/article/1259681-overview#showall
https://www.physiotherapy-treatment.com/congenital-vertical-talus.html

http://www.chop.edu/conditions-diseases/congenital-vertical-talus

https://www.ncbi.nlm.nih.gov/pubmed/26290087

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2680679/