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Paeds Vivasrheumatology-musculoskeletal-and-sports

Paeds Vivas · rheumatology-musculoskeletal-and-sports

Clubfoot and congenital limb anomalies: Viva

Branching clinical structured oral on the clubfoot and the congenital limb anomalies, covering the four classical deformities and the CAVE mnemonic, the Ponseti method with the percutaneous Achilles tenotomy and the foot-abduction brace, the Pirani and the Dimeglio scores, the idiopathic-versus-syndromic distinction, the relapse and the anterior tibialis tendon transfer, and the classification and the disposition of the congenital limb anomalies.

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Prompt
A newborn boy is found on the routine baby check to have a right foot that is inverted, adducted, and plantarflexed, with a deep medial crease and a small heel in varus, and the deformity does not correct with gentle passive manipulation. The examiner asks how you frame the problem, how you classify the deformity, what the Ponseti method is, what the percutaneous tenotomy is, and how you manage the relapse, with deliberate probes into the syndromic clubfoot and the congenital limb anomalies.

This is a branching oral built to probe the reasoning that holds the four deformities, the Ponseti method, and the brace compliance at the centre, and to expose the candidate who has memorised the headline without the safety-critical corners. The questions escalate from the framing to the classification, the Ponseti method, the tenotomy and the brace, with deliberate probes into the syndromic clubfoot, the relapse, and the congenital limb anomalies. [5]

Opening question: framing the problem

The examiner opens with the rigid, inverted foot at the newborn baby check and asks: how do you frame this problem in a single sentence, and what is your first step? [1]

A strong answer names the idiopathic clubfoot from the rigid, inverted, adducted, and plantarflexed foot, and states that the first step is the exclusion of the syndromic associations and the prompt referral for the Ponseti method within the first week of life. [1][5]

Model answer. This child has the idiopathic clubfoot, from the rigid, inverted, adducted, and plantarflexed foot with the deep medial crease and the small heel in varus. The deformity is structural, not positional, because it does not correct with the passive manipulation. My first step is the full general examination to exclude the syndromic associations — the myelomeningocele, the arthrogryposis, and the other anomalies — and the prompt referral for the Ponseti method within the first one to two weeks of life. [1][10]

Probe one: the classification and the scoring

The examiner presses: describe the four classical deformities, and tell me about the Pirani and the Dimeglio scores. [7]

A strong answer names the CAVE mnemonic, the Pirani six-sign score, and the Dimeglio four-parameter classification. [7]

Model answer. The four deformities are the cavus (the high arch), the adductus (the forefoot turned in), the varus (the heel turned in), and the equinus (the toes down), captured by the CAVE mnemonic in the order the Ponseti method corrects them. The Pirani score assigns zero, half, or one to each of six signs — three hindfoot (the posterior crease, the empty heel, the rigid equinus) and three midfoot (the medial crease, the curved lateral border, the lateral talar head) — for a total of zero to six. A higher Pirani predicts the greater number of casts and the higher likelihood of the tenotomy. The Dimeglio scores four parameters (the sagittal equinus, the frontal varus, the horizontal derotation, the medial adduction) each zero to four, plus four pejorative signs, for a total of zero to twenty. [7][5]

Pitfall probe. How do you tell the positional clubfoot from the idiopathic? The positional clubfoot is flexible and passively correctable to neutral, with no deep creases; the idiopathic is rigid and resists the passive correction. The rigidity is the discriminator. [5]

Probe two: the Ponseti method

The examiner asks: describe the Ponseti method, and tell me the sequence of the correction. [1]

A strong answer names the five to seven weekly long-leg casts, the cavus-adductus-varus-equinus sequence, and the fulcrum at the head of the talus. [1]

Model answer. The Ponseti method is the five to seven weekly long-leg casts that correct the deformities in the cavus, adductus, varus, equinus sequence. The first cast corrects the cavus by elevating the first ray. The casts two to four or five correct the adductus and the varus together by abducting the foot around the head of the talus, the fulcrum, with the counter-pressure on the lateral talar head. The equinus is corrected last by the dorsiflexion. The sequence is biomechanical: the talonavicular joint corrects before the ankle, and a premature dorsiflexion against the tight posterior capsule produces the rocker-bottom foot. [1][3]

Pitfall probe. Why does the talonavicular joint correct before the ankle? Because the abduction around the talus must precede the dorsiflexion, to reduce the navicular and to evert the calcaneus before the ankle is moved. A forced dorsiflexion without the prior correction of the adductus and the varus produces the rocker-bottom foot — the iatrogenic signature of the wrong sequence. [1][5]

Probe three: the tenotomy and the brace

The examiner asks: when do you do the percutaneous tenotomy, and what is the foot-abduction brace protocol? [3]

A strong answer names the tenotomy at eight to ten weeks for the residual equinus and the brace at seventy degrees for twenty-three hours for three months then nightly to four to five years. [3][4]

Model answer. The percutaneous Achilles tenotomy is performed at eight to ten weeks, when the cavus, the adductus, and the varus are corrected but the equinus persists — the Pirani hindfoot score above one. It is performed in eighty to ninety percent of the idiopathic clubfeet, and the post-tenotomy cast is worn for three weeks. The foot-abduction brace is the shoes-and-bar device holding the affected foot at seventy degrees of external rotation and fifteen degrees of dorsiflexion. It is worn twenty-three hours a day for the first three months, then nightly (twelve to fourteen hours) to the age of four to five years. The brace compliance is the single greatest determinant of the long-term success. [1][3]

Pitfall probe. The family stops the brace at eighteen months because the child refuses. What happens, and what is your counselling? The relapse is the likely consequence, with the twenty to thirty percent relapse rate driven largely by the brace non-compliance. The counselling is the explanation of the rationale, the peer support, and the regular review, and the family is the key partner in the compliance. [3][8]

Branch one: the syndromic clubfoot

The examiner pivots: imagine the child has a lumbar skin marker and the asymmetric lower-limb movement. What is this, and how does the management change? [10]

A strong answer names the syndromic clubfoot and the modified Ponseti method. [10]

Model answer. This is the syndromic clubfoot associated with a neural tube defect, most likely a myelomeningocele or an occult spinal dysraphism. The workup includes the spinal ultrasound or the magnetic resonance imaging, and the neurosurgical closure of the myelomeningocele is performed first, with the Ponseti casting begun thereafter, once the wound is healed. The syndromic clubfoot is more rigid and more resistant, with the higher relapse rate, but the modified Ponseti method is still the best first-line. The multidisciplinary spina bifida service coordinates the orthopaedic, the neurosurgical, the urological, and the developmental care. [10][5]

Branch two: the relapse

The examiner pivots again: the child returns at the age of three years with the recurrence of the equinus and the dynamic supination. What is this, and what do you do? [8]

A strong answer names the relapse and the anterior tibialis tendon transfer. [8]

Model answer. This is the relapsed clubfoot, with the dynamic supination from the overpull of the anterior tibialis. The first step is the repeat Ponseti casting, with the five to seven casts and, often, a repeat percutaneous tenotomy. The dynamic supination responds to the anterior tibialis tendon transfer, typically to the lateral cuneiform, performed at the age of two and a half to five years. The more extensive procedures — the posterior release, the posteromedial release — are reserved for the resistant few. [8][5]

Closing question: the congenital limb anomalies

The examiner closes: name the broad classes of the congenital limb anomalies and the disposition. [5]

A strong answer names the longitudinal, transverse, and digital deficiencies and the multidisciplinary disposition. [5]

Model answer. The congenital limb anomalies are classified into the longitudinal deficiencies (the failure of formation across the long axis — the fibular hemimelia, the tibial hemimelia, the proximal femoral focal deficiency), the transverse deficiencies (the failure across the short axis, or the constriction band — the congenital amputation, the amniotic band syndrome), and the digital anomalies (the polydactyly, the syndactyly). The disposition is the early referral to the paediatric orthopaedic surgeon and the prosthetist, the family counselling, and the long-term multidisciplinary care. The candidate who frames the congenital limb anomalies alongside the clubfoot demonstrates the breadth the boards reward. [5]

References

  1. [1]Ponseti IV. Treatment of congenital club foot. J Bone Joint Surg Am, 1992.PMID 1548277
  2. [2]Cooper DM, Dietz FR. Treatment of idiopathic clubfoot. A thirty-year follow-up note. J Bone Joint Surg Am, 1995.PMID 7593056
  3. [3]Morcuende JA, Dolan LA, Dietz FR, Ponseti IV. Radical reduction in the rate of extensive corrective surgery for clubfoot using the Ponseti method. Pediatrics, 2004.PMID 14754952
  4. [4]Jowett CR, Morcuende JA, Ramachandran M. Management of congenital talipes equinovarus using the Ponseti method: a systematic review. J Bone Joint Surg Br, 2011.PMID 21911524
  5. [5]Chu A, Lehman WB. Treatment of Idiopathic Clubfoot in the Ponseti Era and Beyond. Foot Ankle Clin, 2015.PMID 26589078
  6. [7]Canavese F, Dimeglio A. Clinical examination and classification systems of congenital clubfoot: a narrative review. Ann Transl Med, 2021.PMID 34423009
  7. [8]Masrouha K, Chu A, Lehman W. Narrative review of the management of a relapsed clubfoot. Ann Transl Med, 2021.PMID 34423014
  8. [10]Gerlach DJ, Gurnett CA, Limpaphayom N, Alaee F, et al. Early results of the Ponseti method for the treatment of clubfoot associated with myelomeningocele. J Bone Joint Surg Am, 2009.PMID 19487512