🖐️ TOACS FCPS Station · Trident Hand in Achondroplasia

Nelson · 22nd Ed · “Short, broad fingers with a wide gap between the 3rd and 4th digits (trident hand), rhizomelic shortening, frontal bossing, midface hypoplasia”
⏱️ 7 minutes · Examiner-led · Observed station
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📷 Clinical Photograph – Trident Hand

Clinical photograph showing trident hand deformity in achondroplasia
Figure 1 · Trident Hand · Achondroplasia (FGFR3 mutation)

🔍 Key clinical features of Trident Hand:

  • Short, broad fingers – all digits shortened
  • Wide gap between 3rd and 4th digits – “trident” appearance
  • Short, stubby thumbs – often held in a “trident” posture
  • Soft tissue redundancy – fingers appear “fleshy”
  • Associated with rhizomelic shortening (proximal limbs)

📋 Clinical scenario (examiner prompt)

A 6‑month‑old infant is brought with disproportionately short stature (height < 3rd percentile, sitting height normal). The child has a large head with frontal bossing, midface hypoplasia, and a flattened nasal bridge. On examination of the hands, there is a characteristic “trident” appearance – the fingers are short and broad with a prominent gap between the 3rd and 4th digits. The child also has rhizomelic shortening of the arms and legs.

Rhizomelic shortening Trident hand Frontal bossing Midface hypoplasia

🧑‍⚕️ Examiner tasks · TOACS

1. Identify the diagnosis from the clinical image and context.

2. Describe the clinical features (trident hand, rhizomelic shortening, frontal bossing, midface hypoplasia).

3. Explain the underlying genetic abnormality (FGFR3 mutation – achondroplasia).

4. Discuss prognosis and management (multidisciplinary: growth monitoring, respiratory, neurologic, orthopedic).

⚠️ Key concept: A trident hand (short, broad fingers with a wide gap between the 3rd and 4th digits) is a classic feature of achondroplasia. The combination of trident hand, rhizomelic shortening, frontal bossing, midface hypoplasia, and a flattened nasal bridge is highly suggestive. Achondroplasia is caused by a gain-of-function mutation in FGFR3 (autosomal dominant, 80% de novo).

🎯 Expected answers (for examiners)

  • Diagnosis: Achondroplasia (FGFR3 mutation)
  • Clinical features: Trident hand, rhizomelic shortening, frontal bossing, midface hypoplasia, macrocephaly, short stature
  • Genetics: Autosomal dominant (80% de novo) – gain-of-function mutation in FGFR3
  • Prognosis: Near-normal lifespan with proactive management; monitor for foramen magnum stenosis, obstructive sleep apnea, restrictive lung disease, hydrocephalus.

⚡ Quick FCPS‑style MCQ

A 4-year-old with disproportionate short stature, frontal bossing, and a trident hand most likely has:

A. Hypochondroplasia B. Achondroplasia C. Thanatophoric dysplasia D. Osteogenesis imperfecta

📌 Topic summary · Achondroplasia

Gene
FGFR3 (gain-of-function)
Inheritance
Autosomal dominant (80% de novo)
Trident hand
Short, broad fingers; gap between 3rd & 4th digits
Rhizomelic shortening
Proximal limb shortening
Respiratory risk
OSA, restrictive lung disease, central hypoventilation
Management
Multidisciplinary; monitor for foramen magnum stenosis
FeatureAchondroplasia
Incidence1 in 15,000–40,000 live births
CraniofacialFrontal bossing, midface hypoplasia, flattened nasal bridge
HandsTrident hand (short, broad fingers, wide gap between 3rd & 4th)
LimbsRhizomelic shortening (proximal)
NeurologicForamen magnum stenosis, hydrocephalus
PrognosisNear-normal with proactive management
Source: Nelson Textbook of Pediatrics 22nd Ed · Section 467.4: Achondroplasia · TOACS FCPS station.