Case 25: proximal Femroal Focal Deficiency Flashcards
What is proximal femoral facial deficiency (PFFD)
Rare congenial limb deficiency with hypoplasia or absence of the proximal femur
What is the clinical presentation of PFFD
Varies from short femoral segment with a normal acetabulum to a short or absent femur and acetabulum
What is a rotionalplasty
Sx procedure in which the tibia is rotated 180° so that the toes point posteriorly allowing the anatomical ankle to function
What is a syme amputation
Ankle disarticulation , in children with more significant presentation of PFFD
What procedure creates a functional AKA
Syme amputation
What procedure creates a functionalal BKA (transtibial)
Rotationplasty
What is the general PT POC/goals for PFFD
- equalize limb length by height of shoe lift
- suppor the development of symmetrical movements thru PT
- increase strength of involved LE and core
- increased ROM
- WB precautions
- increased indepdennt mobility
What are PT interventions for PFFD
- paient and caregivers training on AROM and PROM, bed mobility , transfers , used of AD , exercises , scar management
- pin and wound care
- general strengthening and conditioning exercises
- HEP
What are the precautions during PT for a child with PFFD (7)
- osteopenia (increased risked for fx)
- pin site infection
- hypertrophic scarring
- loss of ROM
- mm atrophy
- increased risk of falls
- decreased overall conditioning
What are the complications during PT fro a child wiht PFFD (4)
- fx at lengthening site
- pine site infection
- emotional lability related to multiple sx
- fear of movement and falls
What is a rare congenital anomaly characterized by failure of normal development of the proximal femur and hip joint
PFFD
What are the 4 types of classification for PFFD
A: femorla head is present , differentiated by the ° of femoral shortening and disconnection between the head and shaft o femur
B: femoral head is present (same as A)
C: femoral head is absent , differentiated by acetabular dysplasia
D: femoral head is absent , most severe - shortened femur and SA sent of femorla head and acetabulum
What are the unique physcial characteristics that vary depending on the severity for PFFD
- shortened limb
- thick bulbous thigh that has a ships funnel appearance
- abducted and flex femoral segment
What should be contributing factor in the subjects with rotionaplasty when making surgical decisions for child with PFFD
Improved gait pattern
T/F: In individuals with PFFD, those who have rotationplasty demonstrate fewer gait compensations compared to those who have Syme amputation.
True
T/F: Rotationplasty is a viable surgical option for select children with PFFD.
True
The most common first intervention for a 14-month-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
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 and active assisted hip, knee, and ankle ROM, bed mobility and transfers, and weightbearing to tolerance using assistive device
B. Bed mobility and transfers with nonweightbearing gait using assistive device. Hip, knee, and ankle ROM to be initiated one week after surgery during the first acute outpatient physical therapy session and following initiation of lengthening
C. Passive ROM only, bed mobility and transfers, and nonweightbearing 3-point gait
D. Active ROM within pain tolerance, bed mobility and transfers, non- weightbearing 3-point gait until patient is pain-free
A
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 and active assisted hip, knee, and ankle ROM, bed mobility and transfers, and weightbearing to tolerance using assistive device
B. Bed mobility and transfers with nonweightbearing gait using assistive device. Hip, knee, and ankle ROM to be initiated one week after surgery during the first acute outpatient physical therapy session and following initiation of lengthening
C. Passive ROM only, bed mobility and transfers, and nonweightbearing 3-point gait
D. Active ROM within pain tolerance, bed mobility and transfers, non- weightbearing 3-point gait until patient is pain-free
