Eric Heimberger, M.D., Resident Orthopaedic Surgery
S. Jay Kumar, M.D., Attending Pediatric Orthopaedic Surgery
September 25, 1995
CLINICAL CASE PRESENTATION
THE ALFRED I. DUPONT INSTITUTE
HISTORY: This is a 1+3 year old female who was the product of a NSVD at 38 weeks gestation without complications. She was one of twins with her sister healthy and without deformity. Soon after birth the patient was noted to have a severe deformity involving the right leg and right foot. Radiographs at that time revealed absence of the fibula on the right with ipsilateral hypoplasia of the femur. There is no family history of similar deformities and no identifiable environmental insult during pregnancy. She is otherwise healthy.
PHYSICAL EXAM: Examination of the right lower extremity demonstrates shortening of the right thigh with a 10 degree flexion contracture present in the knee. The knee is noted to be in valgus. There is moderate anteromedial bowing present in the leg with a dimple present over the anterior aspect of the tibia at the apex of the bow. The foot is held in a position of severe equinovalgus with absence of the lateral two rays noted. There is syndactyly present among the remaining digits. Circulation is normal and skin condition is good. Overall, the right lower extremity is noted to be 5 cm. shortened when compared to the contralateral normal extremity. Attempts at ambulation were limited by the severe deformity present in the right lower extremity.
RADIOGRAPHS: Absent right fibula and lateral two rays of right foot with moderate anteromedial bowing of tibia. The right femur was also noted to be shortened.
TREATMENT: The patient initially underwent a corrective tibial osteotomy and Boyd amputation of right foot. The postoperative course was complicated by the development of a nonunion at the osteotomy site necessitating resection of the pseudarthrosis with placement of K-wires. Following this, she did well ambulating with the use of a below-knee prosthesis.
FOLLOW-UP: At 9 years of age the patient was noted to have an increased flexion contracture about the knee with radiographs demonstrating a recurrent valgus deformity. She subsequently underwent a repeat tibial derotational osteotomy with correction of the valgus deformity. Her postoperative course was uncomplicated. Currently she is 17 years old and ambulates well with the use of a custom below-knee PTB prosthesis with a carbon copy foot.
There have been many different classifications developed for CLDF.
Achterman & Kalamchi's classification is most commonly used and will be described.
This classification is useful as a prognostic indicator and for aiding the decision-making process regarding treatment.
1) predicted LLI greater than 3 inches
2) severely deformed foot that cannot be surgically corrected
4) psychologic inability to cope with multiple procedures
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