Updated: 5/25/2021

Radial Clubhand (radial deficiency)

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https://upload.orthobullets.com/topic/6067/images/TAR - xray - colorado_moved.jpg
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https://upload.orthobullets.com/topic/6067/images/clinical photo - colorado_moved.jpg
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https://upload.orthobullets.com/topic/6067/images/Case A - clinical photo - Kosin_moved.jpg
https://upload.orthobullets.com/topic/6067/images/xray - colorado_moved.jpg
  • Summary
    • Radial Clubhand is a rare birth defect characterized by a deformity of hand, with perpendicular relationship between forearm and wrist, and an absent thumb. 
    • Diagnosis is made on physical exam.
    • Treatment may be observation or surgical reconstruction when the child is around 6 months of age. 
  • EPIDEMIOLOGY
    • Incidence
      • rare
        • 1 per 100,000
      • anatomic location
        • bilateral in 50-72%
        • thumb usually deficient as well
  • Etiology
    • Genetics
      • likely related to sonic hedgehog gene
    • Associated with
      • TAR
        • autosomal recessive condition with thrombocytopenia and absent radius
        • different in that thumb is typically present
      • Fanconi's anemia
        • autosomal recessive condition with aplastic anemia
        • Fanconi screen and chromosomal breakage test to screen
        • treatment is bone marrow transplant
      • Holt-Oram syndrome
        • autosomal dominant condition characterized by cardiac defects
      • VACTERL Syndrome
        • vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula, renal agenesis, and limb defects)
      • VATER Syndrome
        • vertebral anomalies, anal atresia, tracheoesophageal fistula, esophageal atresia, renal agenesis)
  • Classification
    • Bayne and Klug Classification
      Type I
      Deficient distal radial epiphysis
      Type II
      Deficient distal and proximal radial epiphyses
      Type III
      Present proximally (partial aplasia)
      Type IV
      Completely absent (total aplasia - most common)
  • Presentation
    • Physical exam
      • deformity of hand with perpendicular relationship between forearm and wrist
      • absent thumb
      • perform careful elbow examination
  • Imaging
    • Radiographs
      • entire radius and often thumb is absent
    • Laboratory
      • must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions
  • Treatment
    • Nonoperative
      • passive stretching
        • target tight radial-sided structures
      • observation
        • indicated if absent elbow motion or biceps deficiency
          • hand deformity allows for extra reach to mouth in presence of a stiff elbow
    • Operative
      • hand centralization
        • indications
          • good elbow motion and biceps function intact
          • done at 6-12 months of age
          • followed by tendon transfers
        • contraindications
          • older patient with good function
          • patients with elbow extension contracture who rely on radial deviation
          • proximate terminal condition
        • technique
          • involves resection of varying amount of carpus, shortening of ECU, and, if needed, an angular osteotomy of the ulna (be sure to spare ulnar distal physis)
          • may do as two stage procedure in combination with a distraction external fixator
          • if thumb deformity then combine with thumb reconstruction at 18 months of age

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Flashcards (5)
Cards
1 of 5
Questions (6)

(OBQ11.143) A child is seen in the pediatric orthopedic hand clinic for evaluation of a congenital deformity. A clinical photograph and radiograph are seen in Figures A and B. What is the next best step in this child's evaluation to rule out an associated autosomal-recessive lethal condition?

QID: 3566
FIGURES:
1

Cardiac ultrasound and renal ultrasound

30%

(972/3261)

2

Cardiac ultrasound, barium swallow and MRI

3%

(102/3261)

3

LFTs, CBC and cardiac ultrasound

7%

(242/3261)

4

Cardiac ultrasound, peripheral blood smear and MRI

9%

(282/3261)

5

CBC, peripheral blood smear and chromosomal breakage analysis

50%

(1645/3261)

L 4 C

Select Answer to see Preferred Response

(OBQ11.149) A 7-month-old boy has radial longitudinal deficiency. The initial work-up is negative for any cardiac, hematopoetic or renal abnormalities. He has good active elbow flexion and no other deformities. A clinical image and radiograph are seen in Figures A and B. Surgical management should include which of the following?

QID: 3572
FIGURES:
1

Definitive splinting and stretching

8%

(323/4028)

2

Pollicization of the index finger

3%

(109/4028)

3

ECRB and ECRL transfers to the ulna

2%

(96/4028)

4

Free fibula transfer to the forearm

3%

(129/4028)

5

Ulna centralization and possible tendon transfers

83%

(3336/4028)

L 2 C

Select Answer to see Preferred Response

(OBQ09.152) A 6-year-old boy presents to your office with a forearm deformity and the radiographs shown in Figure A. What additional tests must be ordered for this patient?

QID: 2965
FIGURES:
1

CBC

2%

(93/4541)

2

CBC and renal ultrasound only

4%

(193/4541)

3

CBC, renal ultrasound, and echocardiogram

92%

(4156/4541)

4

CBC and MRI

1%

(28/4541)

5

No additional testing needed

1%

(45/4541)

L 1 D

Select Answer to see Preferred Response

(OBQ08.19) A 2-year-old boy has the upper limb deformity seen in Figures A and B. All of the following are associated with this condition EXCEPT?

QID: 405
FIGURES:
1

Fanconi's Anemia

5%

(125/2556)

2

Holt-Oram syndrome

4%

(96/2556)

3

VATER syndrome

3%

(74/2556)

4

VACTERL syndrome

3%

(77/2556)

5

Osteogenesis Imperfecta

85%

(2173/2556)

L 1 D

Select Answer to see Preferred Response

(SAE07PE.82) A 4-year-old child was born with bilateral congenital radial clubhands. Which of the following associated conditions is a contraindication to centralization of the hands on the ulna?

QID: 6142
1

Congenital scoliosis

0%

(1/713)

2

Hypoplastic thumb

21%

(151/713)

3

Tracheoesophageal fistula

1%

(8/713)

4

Imperforate anus

1%

(6/713)

5

Lack of elbow flexion

76%

(542/713)

L 2 E

Select Answer to see Preferred Response

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