Congenital Tibial Deficiency:
The TSRH Experience
Rebecca Clinton, M.D.
John Birch, M.D., FRCS(C)
Mikhail L. Samchukov, M.D.
Alexander Cherkashin, M.D
Texas Scottish Rite Hospital for Children
Dallas, Texas
Disclosures
Rebecca Clinton, MD – None
J. Birch, M. Samchukov, A. Cherkashin:
Royalties through our employer
(TSRH) from Orthofix for a circular
fixator (True-lok).
Jones classification JBJS 1978
Four Morphological
Types:
IA: No proximal tibia
IB: Proximal tibia
unossified at birth
(cartilaginous anlage)
II: No distal tibia
III: Distal tibia (only)
IV: Diastasis
63 patients, 95 limbs
Trying to address those patients whose
limbs don’t fit within Jones classification
Purpose
•  To describe patients with congenital
tibial deficiency treated at TSRH
between 1975-present
•  Chart and Radiographic Review:
– Jones type
– Treatment elected
– Catalogue associated anomalies
Patient population
•  Final population
– 95 patients, 125 limbs
– 30 bilateral (32%)
– 75/95 patients with associated anomalies
(79%)
Distribution by Jones Type
Type IA: 53 (73 limbs)
Type IB: 6 (6 limbs)
Type II: 18 (18 limbs)
Type III:
2 (2 limbs)
Type IV: 11 (12 limbs)
Distribution by Jones Type
Type IA: 53 (73 limbs)
Type IB: 6 (6 limbs)
Type II: 18 (18 limbs)
Type III:
2 (2 limbs)
Type IV: 11 (12 limbs)
TBD:
14 (14 limbs)
Type 1A
•  53 patients, 73 limbs
•  Treatment:
– 63/73 ultimately treated with knee disarticulation
• 47 primary
• 16 staged (3 after Syme, 13 after Brown)
Type 1A
10/73 NOT Disarticulation (yet):
2 Syme and knee fusion
3 unrevised failed Brown’s with knee contractures
5 no surgery (non-ambulatory, or pending)
Brown Procedure in Type 1A:
•  Total of 17 Brown procedures performed
•  13 subsequent knee disarticulations
•  3 other failures with significant
contractures
•  1 non-ambulatory patient
Brown Procedure in Type 1A:
•  Total of 17 Brown procedures performed
•  13 subsequent knee disarticulations
•  3 other failures with significant
contractures
•  1 non-ambulatory patient
= Unqualified Failure
•  Total of 17 Brown procedures performed
•  13 subsequent knee disarticulations
•  3 other failures with significant contractures
•  1 non ambulatory patient
Type 1B: Proximal Tibia Unossified at
Birth
6 patients (6 limbs)
Treatment:
• 3 Syme and tib/fib
synostosis
• 1 Syme
• 1 knee
disarticulation
• 1 pending
Type 2: Ossified Proximal Tibia
Type 2: 18 patients, 18 limbs
11 Syme and tib/fib
synostosis
3 knee disarticulation
3 Syme
1 BKA with fibular
excision
Type 3: Distal (Only) Tibia
Type 3: Distal (Only) Tibia
•  2 Patients (2 limbs)
•  Treatment:
-1 Syme and tib/fib synostosis
-1 knee disarticulation
TYPE III?
•  At 13 months, proximal tibia ossifies
•  At 13 months, proximal tibia ossifies
•  We had NO CASES of “true” Type III
Type 4: Tibio-Fibular Diastasis
Type 4:11 patients, 12 limbs
8 Syme
1 tib/fib synostosis
3 Foot salvage, limb
reconstruction
TBD: The Ones That Didn’t “Fit”
-14 patients/limbs
-Global tibial
dysplasia/proximal
and distal fibular
overgrowth
-14 patients/limbs
-Global tibial
dysplasia/proximal
and distal fibular
overgrowth
-Spectrum of
deformity
A Spectrum of Treatment:
5 No surgery required
1 duplicated great toe excision
1 rotational osteotomy,
duplicated great toe excision
3 Reconstruction/Lengthening
4 Amputation (3 Syme,
1 knee disarticulation)
8/14 had great toe duplication/hypoplasia,
representing 8 of the 15 seen in the 125 limbs
in this population.
How To Classify?
Type I
– Weber:
Type IV A Type V A
How To Classify?
Type I
Type IV A Type V A
– Weber:
– “Modify” Jones, adding a “Type
V” (global tibial deficiency, proximal and
distal present)
Summary
Type 1A Type 1B Type 2
Type 3
Type 4
Type 5
Patients
53
6
18
2
11
14
Limbs
73
6
18
2
12
14
• 
• 
• 
• 
• 
1A: 63/73 knee disarticulation
1B/2 and 2: Syme and tib/fib synostosis mostly
No true III: proximal epiphysis appeared
IV: mostly Syme
V: A spectrum from no treatment to amputation
Brown procedure is
ineffective surgery
– In keeping with
results from prior
studies
Thank you!