FRCS Paediatric Ortho — improved notes
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020. PFFD / proximal femoral focal deficiency

visual-inline rebuild1 extracted figuresspeaker notes XML70 study lines8 Q&A

Individual slide images have been cropped from the rendered slide and inserted beside the matching note section. The full slide remains below only for layout/audit.

Clean FRCS notes with inline figures

PFFD

•Proximal femoral focal deficiency
•Clinical photo of child with significant LLD
•Femurs appears short, flexed + abducted + ER (FABER)
•Knee + foot abnormalities
•Facial features (AD type)
•AP Xray pelvis with hip abnormalities + short femur + coxa vara

Epiphysis present?

•Acetabular (AI) / tear drop abnormalities
•Look for associated deformities (fibular hemimelia)
•Impression: PFFD, Aikten C, but need to have MRI to see if femoral ossification center is present

History

Birth history

•Maternal thalidomide

Examination

Clinical photograph - PFFD clinical photograph
Fig 1. Clinical photograph - PFFD clinical photographtop centre
•Hip
•Knees stability

Foot functional?

•LLD (at least to mid tibia for it to be salvageable / reconstructable)
•MRI x assessment of development (any femoral head) and version

Management

•Treatment aim is to allow the patient to ambulate, whether this is through patient’s native limb or by prosthesis
•Factors to consider (LAF)
•LLD

Aitken classification (hip)

•Foot stability
•Conservative (SHOERAISE)
•First thing to determine is whether the LLD will be too significant for reconstructive procedures
•Predict LLD >20cm at maturity
•Foot to level of mid tibia
•Femur <50% of other leg
•If LLD not significant, then look to see if the hip is reconstructable.

Aitken classification

•A and B – femoral head present = reconstruct hip (valgus derotational osteotomy, PAO, ITB release, excise pseudoarthrosis + BG)
•C and D – femoral head absent – iliofemoral fusion
•Then followed by limb lengthening procedures
•If LLD significant, then the aim is for prosthesis fitting. See if foot is functional or not
•If functional, then iliofemoral fusion + van nes rotationoplasty
•If non functional, then knee fusion + syme amputation
•Aitken classification, A-D based on femoral and acetabular involvement
•AB femoral head +, CD femoral head -
•A→D head present, subtro pseudoarthrosis, absent head, absent head and proximal femur
•A→D Acetabular normal, mild dysplasia, severe dysplasia, abs

Lengthening timing

See LLD amount, ?need staged disease, how many times does it take?

•Roughly 1cm /month
•Consider psychological factors, roughly can start at 5y

Complication of lengthening

•Soft tissue: NV
•Bone: non union
•Joint: contracture
•General: infection
•Lengthening max 20-30%

Related to complications

What is the pathology?

•Sclerotome subtraction

Other classifications

Gillespie classification

•I congenital short femur (40-60%), functional hip and knee
•II true PFFD: defect + short, hip dysplasia, non functional knee
•Other associated anatomical anomalies
•Hip – coxa vara + retroversion, dysplasia
•Femur – PFFD
•Knee – LFC hypoplasia, genu valgus, patellar instability, cruciate insufficiency
•Tibia: Short + AM bowing
•Fib: fibular hemimelia
•Ankle: ankle valgus + ball and socket joint
•Foot: tarsal coalition, absent fib ray

Direct Q&A

Q1 Epiphysis present?

Acetabular (AI) / tear drop abnormalities

•Look for associated deformities (fibular hemimelia)
•Impression: PFFD, Aikten C, but need to have MRI to see if femoral ossification center is present
Q2 What examination findings should be assessed in PFFD / proximal femoral focal deficiency?

Hip

•Knees stability
Q3 Foot functional?
•LLD (at least to mid tibia for it to be salvageable / reconstructable)
•MRI x assessment of development (any femoral head) and version
Q4 How is PFFD / proximal femoral focal deficiency managed?
•Treatment aim is to allow the patient to ambulate, whether this is through patient’s native limb or by prosthesis
•Factors to consider (LAF)
•LLD

Aitken classification (hip)

•Foot stability
•Conservative (SHOERAISE)
•First thing to determine is whether the LLD will be too significant for reconstructive procedures
•Predict LLD >20cm at maturity
•Foot to level of mid tibia
•Femur <50% of other leg
•If LLD not significant, then look to see if the hip is reconstructable.
Q5 How is PFFD / proximal femoral focal deficiency classified here?
•Foot stability
•Conservative (SHOERAISE)
•First thing to determine is whether the LLD will be too significant for reconstructive procedures
•Predict LLD >20cm at maturity
•Foot to level of mid tibia
•Femur <50% of other leg
•If LLD not significant, then look to see if the hip is reconstructable.
Q6 See LLD amount, ?need staged disease, how many times does it take?
•Roughly 1cm /month
•Consider psychological factors, roughly can start at 5y
Q7 What complications are listed for PFFD / proximal femoral focal deficiency?

Soft tissue: NV

•Bone: non union
•Joint: contracture
•General: infection
•Lengthening max 20-30%
Q8 What is the pathology?

Sclerotome subtraction

Raw speaker-note hierarchy audit

#lvlmarLindentlogicaloriginal note line
10NoneNone0PFFD
20NoneNone0Proximal femoral focal deficiency
30NoneNone0Clinical photo of child with significant LLD
40NoneNone0Femurs appears short, flexed + abducted + ER (FABER)
50NoneNone0Knee + foot abnormalities
60NoneNone0Facial features (AD type)
70NoneNone0AP Xray pelvis with hip abnormalities + short femur + coxa vara
80NoneNone0Epiphysis present?
90NoneNone0Acetabular (AI) / tear drop abnormalities
100000Look for associated deformities (fibular hemimelia)
110NoneNone0Impression: PFFD, Aikten C, but need to have MRI to see if femoral ossification center is present
120NoneNone0Hx:
130NoneNone0Birth history
140NoneNone0Maternal thalidomide
150NoneNone0PE:
160NoneNone0Hip
170NoneNone0Knees stability
180NoneNone0Foot functional?
190NoneNone0LLD (at least to mid tibia for it to be salvageable / reconstructable)
200000MRI x assessment of development (any femoral head) and version
210NoneNone0Mx
220NoneNone0Treatment aim is to allow the patient to ambulate, whether this is through patient’s native limb or by prosthesis
230NoneNone0Factors to consider (LAF)
241685800-2286002LLD
251685800-2286002Aitken classification (hip)
261685800-2286002Foot stability
270000Conservative (SHOERAISE)
280000First thing to determine is whether the LLD will be too significant for reconstructive procedures
290171450-1714501Predict LLD >20cm at maturity
300171450-1714501Foot to level of mid tibia
310171450-1714501Femur <50% of other leg
320000If LLD not significant, then look to see if the hip is reconstructable.
330000Aitken classification
340171450-1714501A and B – femoral head present = reconstruct hip (valgus derotational osteotomy, PAO, ITB release, excise pseudoarthrosis + BG)
350171450-1714501C and D – femoral head absent – iliofemoral fusion
360171450-1714501Then followed by limb lengthening procedures
370000If LLD significant, then the aim is for prosthesis fitting. See if foot is functional or not
380171450-1714501If functional, then iliofemoral fusion + van nes rotationoplasty
390171450-1714501If non functional, then knee fusion + syme amputation
400NoneNone0=========================
410NoneNone0Aitken classification, A-D based on femoral and acetabular involvement
420NoneNone0AB femoral head +, CD femoral head -
430171450-1714501A->D head present, subtro pseudoarthrosis, absent head, absent head and proximal femur
440171450-1714501A→D Acetabular normal, mild dysplasia, severe dysplasia, abs
450NoneNone0Lengthening timing
461NoneNone1See LLD amount, ?need staged disease, how many times does it take?
471NoneNone1Roughly 1cm /month
481NoneNone1Consider psychological factors, roughly can start at 5y
490NoneNone0Complication of lengthening
501NoneNone1Soft tissue: NV
511NoneNone1Bone: non union
521NoneNone1Joint: contracture
531NoneNone1General: infection
540NoneNone0Lengthening max 20-30%
550NoneNone0Related to complications
560NoneNone0===============================
570NoneNone0What is the pathology?
580NoneNone0Sclerotome subtraction
590NoneNone0Other classifications
600NoneNone0Gillespie classification
610NoneNone0I congenital short femur (40-60%), functional hip and knee
620NoneNone0II true PFFD: defect + short, hip dysplasia, non functional knee
630000Other associated anatomical anomalies
641628650-1714502Hip – coxa vara + retroversion, dysplasia
651628650-1714502Femur – PFFD
661628650-1714502Knee – LFC hypoplasia, genu valgus, patellar instability, cruciate insufficiency
671628650-1714502Tibia: Short + AM bowing
681628650-1714502Fib: fibular hemimelia
691628650-1714502Ankle: ankle valgus + ball and socket joint
701628650-1714502Foot: tarsal coalition, absent fib ray

Improved semantic hierarchy used for the study notes

#semantic levelsection?rewritten line
10PFFD
21Proximal femoral focal deficiency
31Clinical photo of child with significant LLD
41Femurs appears short, flexed + abducted + ER (FABER)
51Knee + foot abnormalities
61Facial features (AD type)
71AP Xray pelvis with hip abnormalities + short femur + coxa vara
80yesEpiphysis present?
91Acetabular (AI) / tear drop abnormalities
101Look for associated deformities (fibular hemimelia)
111Impression: PFFD, Aikten C, but need to have MRI to see if femoral ossification center is present
120yesHistory
130yesBirth history
141Maternal thalidomide
150yesExamination
161Hip
171Knees stability
180yesFoot functional?
191LLD (at least to mid tibia for it to be salvageable / reconstructable)
201MRI x assessment of development (any femoral head) and version
210yesManagement
221Treatment aim is to allow the patient to ambulate, whether this is through patient’s native limb or by prosthesis
231Factors to consider (LAF)
242LLD
252yesAitken classification (hip)
263Foot stability
270Conservative (SHOERAISE)
280First thing to determine is whether the LLD will be too significant for reconstructive procedures
293Predict LLD >20cm at maturity
301Foot to level of mid tibia
313Femur <50% of other leg
320If LLD not significant, then look to see if the hip is reconstructable.
330yesAitken classification
341A and B – femoral head present = reconstruct hip (valgus derotational osteotomy, PAO, ITB release, excise pseudoarthrosis + BG)
351C and D – femoral head absent – iliofemoral fusion
361Then followed by limb lengthening procedures
371If LLD significant, then the aim is for prosthesis fitting. See if foot is functional or not
381If functional, then iliofemoral fusion + van nes rotationoplasty
391If non functional, then knee fusion + syme amputation
400——
411Aitken classification, A-D based on femoral and acetabular involvement
421AB femoral head +, CD femoral head -
431A→D head present, subtro pseudoarthrosis, absent head, absent head and proximal femur
441A→D Acetabular normal, mild dysplasia, severe dysplasia, abs
450yesLengthening timing
460yesSee LLD amount, ?need staged disease, how many times does it take?
471Roughly 1cm /month
481Consider psychological factors, roughly can start at 5y
490yesComplication of lengthening
501Soft tissue: NV
511Bone: non union
521Joint: contracture
531General: infection
541Lengthening max 20-30%
550yesRelated to complications
560——
570yesWhat is the pathology?
581Sclerotome subtraction
590yesOther classifications
600yesGillespie classification
611I congenital short femur (40-60%), functional hip and knee
621II true PFFD: defect + short, hip dysplasia, non functional knee
631Other associated anatomical anomalies
642Hip – coxa vara + retroversion, dysplasia
652Femur – PFFD
662Knee – LFC hypoplasia, genu valgus, patellar instability, cruciate insufficiency
672Tibia: Short + AM bowing
682Fib: fibular hemimelia
692Ankle: ankle valgus + ball and socket joint
702Foot: tarsal coalition, absent fib ray
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