Case 25: Congenital Limb Deficiency - Proximal Femoral Focal Deficiency (exam 3?) Flashcards
What is Proximal femoral focal deficiency (PFFD)?
A rare congenital anomaly characterized by failure of normal development of the proximal femur and
hip joint.
The clinical classification is defined by the degree of hip involvement and femoral shortening.
FEMORAL OSTEOTOMY
Surgical procedure to restore normal anatomy of
the hip and provide optimal joint alignment.
____ ________ is the process of growing new bone and soft tissue through gradually lengthening the shortened limb.
Most common method is the application of an external fixator device that incorporates thin wires,
thicker pins, and pins with screws to provide gradual distraction.
LIMB LENGTHENING
_______ is a surgical procedure in which the tibia is rotated 180° so that the toes point posteriorly allowing the anatomical ankle to function as
the knee in order to create a below-knee amputation level.
ROTATIONPLASTY
Precautions during physical therapy:
- osteopenia increasing risk for fractures
- pin site infection
- hypertrophic scarring
- loss of ROM
- muscle atrophy
- increased risk of falls
- decreased overall conditioning
Complications during physical therapy:
- fractures at lengthening site
- pin site infection
- emotional lability related to multiple surgeries
- fear of movement and falls
General PT POC/Goals:
- monitor limb length discrepancy with recommendations for appropriate height of shoe lift
- increase strength of involved LE and core
- increase ROM in joints above and below during limb lengthening
- maintain weightbearing precautions
- increase independent functional mobility with AD as indicated throughout each phase of intervention
What is the clinical classification of PFFD defined by?
degree of hip involvement and femoral shortening
The most commonly cited classification is the Aitken classification which classifies PFFD into four types: A-D.
Describe types A and B.
A & B - femoral head is present
A & B are differentiated by degree of femoral shortening & disconnection between the head and shaft of the femur - known as pseudoarthrosis
Describe Aitken classification types C & D.
C & D - femoral head is absent
- The degree of acetabular dysplasia differentiates types C & D.
What is the most severe form of PFFD with significantly shortened femur and absent femoral head and acetabulum?
Type D
What are some characteristics of PFFD?
- shortened limb
- thick bulbous thigh that has a ship’s funnel appearance
- abducted and flexed femoral segment
- possibly a longitudinal deficiency of the fibula
What are some associated impairments of PFFD?
- knee instability with absence of the cruciate ligaments
- genu valgus
- fibula deficiency w/ talocalcaneal coalition and foot deformity
- bilateral PFFD
- associated upper limb anomalies
What are the three main surgical options for individuals with PFFD?
- femoral osteotomy with staged limb lengthenings
- femoral osteotomy, knee fusion, Syme amputation
- femoral osteotomy with tibia rotationplasty
Congenital Limb Deficiency: PFFD
What is the most appropriate inpatient physical therapy intervention for a 6 year old male immediately after limb lengthening and application of unilateral external fixator?
A. Active & active assisted hip, knee, and ankle ROM, bed mobility and transfers, &. weightbearing to tolerance using AD
B. Bed mobility and transfers with nonweightbearing gait using AD. Hip, knee, and ankle ORM initiated one week after surgery during the first acute OP PT session
C. Passive ROM only, bed mobility and transfers, and nonweightbearing 3-point gait
D. Active ROM within pain tolerance, bed mobility and transfers, nonweightbearing 3-point gait until patient is pain-free
A. Active & active assisted hip, knee, and ankle ROM, bed mobility and transfers, &. weightbearing to tolerance using AD
-early weigthbearing is important for bone healing
Congenital Limb Deficiency: PFFD
The most common first intervention for a 14month old toddler with left Aitken Type C PFFD is:
A. Surgical amputation of the left foot with knee fusion
B. Shoe lift with AFO
C. Extension prosthesis with prosthetic knee
D. Femoral osteotomy with knee fusion
C. Extension prosthesis with prosthetic knee
- equalizing limb lengths & offering the patient knee function is important for normalizing development allowing toddler to transition to into and out of sitting and standing
