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OR12.1 | Congenital Lesions — PBL Case

CLINICAL SETTING

Priya, a 2-week-old female neonate, is brought to the paediatric orthopaedic outpatient department by her parents. She was born at term by normal vaginal delivery after a breech presentation. Her mother noticed that her right foot 'looks turned inward and downward' and cannot be straightened, and that one of her legs appears shorter than the other. There is no family history of musculoskeletal disease. Examination reveals a rigid right foot held in plantarflexion, inversion, adduction, and supination. The deformity cannot be passively corrected to neutral. There is also asymmetric thigh skin folds on the left side with a positive Galeazzi sign (left knee lower). On hip examination of the left hip, Ortolani test elicits a palpable clunk on abduction.

Trigger 1: The Foot Deformity

The orthopaedic resident examines Priya's right foot. The deformity is rigid, involving the hindfoot and forefoot. The heel is not palpable in its normal position. The skin on the medial aspect is creased. The Pirani score is 5.5/6 (severe). The mother asks: 'Will her foot ever look normal, and will she be able to walk?'

DISCUSSION POINTS

  • Name the four components of the right foot deformity using the CAVE mnemonic and explain what each component means anatomically.
  • What is the Pirani score and what does a score of 5.5/6 indicate about severity and likely treatment?
  • What is the Ponseti method? Describe the sequence of cast changes and the role of percutaneous Achilles tenotomy in this case.
  • How would you counsel Priya's mother regarding prognosis, relapse risk, and the role of the foot abduction brace (FAB) after casting?
Click to reveal Trigger 2: The Abnormal Hip (discuss previous trigger first!)

Trigger 2: The Abnormal Hip

Attention turns to Priya's left hip. Ortolani test is unequivocally positive (palpable reduction clunk). Barlow test on the left produces a subtle posterior subluxation. An ultrasound of both hips is arranged. The ultrasound report reads: 'Right hip normal, Graf Type I (alpha angle 63°). Left hip: femoral head coverage reduced, acetabular roof poorly formed, alpha angle 43° — Graf Type III, consistent with dislocated hip.'

DISCUSSION POINTS

  • Explain why the alpha angle of 43° confirms dislocation and what Graf Type III signifies in terms of bony and cartilaginous acetabular development.
  • Why is plain X-ray not used for hip evaluation in a 2-week-old and at what age does it become the standard imaging modality?
  • Outline the immediate management plan for the dislocated left hip. What position does the Pavlik harness maintain, and what is the mechanism by which it promotes hip stability?
  • What is the most serious complication of DDH treatment and how is it prevented during Pavlik harness application?
Click to reveal Trigger 3: Complex Congenital Presentation — Prioritisation and Long-Term Follow-Up (discuss previous trigger first!)

Trigger 3: Complex Congenital Presentation — Prioritisation and Long-Term Follow-Up

At 6-month review, Priya's CTEV has been treated with 6 Ponseti casts; after percutaneous Achilles tenotomy, the right foot is now in a plantigrade position. She is in a foot abduction brace (Denis Browne). Her left hip Pavlik harness treatment has been ongoing for 10 weeks; repeat USG shows Graf Type II (alpha angle 55°) — improving but not yet normal. The father now raises a new concern: the family's second cousin had scoliosis that required surgery, and he asks whether Priya is at risk.

DISCUSSION POINTS

  • What does Graf Type II at 10 weeks indicate and how should the Pavlik harness treatment be modified or continued?
  • A follow-up X-ray of the pelvis at 6 months shows irregular ossification of the left femoral head capital epiphysis. What complication does this suggest and what are its causes in DDH treatment?
  • What are the risk factors for adolescent idiopathic scoliosis (AIS) and how does the screening examination (Adam's forward bend test + scoliometer) work? At what Cobb angle thresholds do you observe, brace, or operate?
  • Integrate the management of Priya's two conditions: if she develops a significant scoliosis at age 12 in addition to a sequela of DDH, how do the treatment priorities interact? Discuss the multidisciplinary approach.

Learning Issues

Research these questions and bring your findings to the discussion.

  1. [OR12.1] What are the clinical features, grading systems (Pirani score, Graf USG classification, Cobb angle), and evidence-based management principles for each of the four major congenital orthopaedic conditions: CTEV, DDH, congenital muscular torticollis, and scoliosis/spina bifida?
  2. [OR12.1] What is the Ponseti method for CTEV? Describe the step-by-step sequence, the role of percutaneous Achilles tenotomy, the foot abduction brace protocol, and the management of relapse.
  3. [OR12.1] How is DDH screened (Ortolani, Barlow, Galeazzi tests), investigated (USG < 6 months, X-ray > 6 months), and managed (Pavlik harness → closed reduction → open reduction)? What is avascular necrosis of the femoral head and how is it prevented?
  4. [OR12.1] What are the Cobb angle thresholds for observation, bracing, and surgery in adolescent idiopathic scoliosis? How does the neurogenic scoliosis of spina bifida differ from AIS in management?