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Congenital longitudinal deficiency of the tibia
P L Schoenecker1, A M Capelli, E A Millar
1Shriners Hospital for Crippled Children, St. Louis, Missouri 63131.
The Journal of Bone and Joint Surgery. American Volume
|February 1, 1989
Summary
Most patients with tibial hemimelia achieved independent walking after surgery. Ablative procedures, including Syme amputation, were common, with limb salvage considered for less severe cases.
Area of Science:
- Orthopedic surgery
- Pediatric orthopedics
- Congenital limb differences
Background:
- Congenital longitudinal deficiency of the tibia, or tibial hemimelia, presents a significant challenge in pediatric limb development.
- Classification systems, such as that by Jones et al., aid in categorizing the severity of tibial hemimelia.
- Treatment strategies aim to improve function and ambulation in affected individuals.
Purpose of the Study:
- To retrospectively analyze the outcomes of surgical management for tibial hemimelia.
- To evaluate the effectiveness of different ablative procedures and limb salvage techniques based on radiographic classification.
- To assess long-term functional results, particularly independent walking, in patients with tibial hemimelia.
Main Methods:
- Retrospective review of 57 patients (71 limbs) with congenital tibial hemimelia.
- Classification of limbs into Types 1-4 based on the Jones et al. radiographic system.
- Analysis of surgical interventions, including knee disarticulation, Syme amputation, Chopart amputation, and fibular transfer (Brown procedure).
- Evaluation of functional outcomes, focusing on independent ambulation at an average 9-year follow-up.
Main Results:
- Fifty-six of 57 patients achieved independent walking.
- Ablative procedures were performed on 61 of 71 limbs.
- Type 1 or 2 deficiencies (54 limbs) often resulted in knee disarticulation (22) or Syme amputation (25).
- The Brown procedure for medial fibular transfer had suboptimal results, requiring further operations in 10 of 14 limbs.
- Type 3 or 4 deficiencies (17 limbs) predominantly underwent Syme amputation (9) or Chopart amputation (4), with limb salvage attempted in some cases.
Conclusions:
- Independent ambulation is achievable for the majority of patients with tibial hemimelia, even after ablative surgery.
- Syme amputation is frequently necessary for Type 4 deficiencies due to projected leg-length discrepancies.
- Limb salvage and reconstructive techniques, like the Brown procedure, may have limited success and require further interventions.