Slide 024 — Congenital tibial pseudoarthrosis / anterolateral tibial bowing

Clean FRCS notes with inline figures

•Clinically photo showing patient’s right leg with abnormal angulation at level of distal tibia, with apex anterior, on frontal view there is no significant angulation (AL bowing), multiple hyperpigmented skin lesions ?scar/ café au lait spots

•Any neurofibroma / axillary freckles

•Anterior bowing of tibia is likely pathological

•Xray showing abnormalities of distal tibia and fibula, # with ?pseudoarthrosis over tibia, fibula length is normal → Dx Tibial pseudoarthrosis

Differential diagnosis

•NF with tibial pseudoarthrosis

•Tibial hemimelia (anterolateral bowing)

•Fibrous dysplasia

•Trauma with fracture callus

•Tumor (EWS, osteofibrous dysplasia, adamantinoma)

•Osteomyelitis

Crawford classification. Others: Anderson, Boyd

Type / group	Definition / note
I	Dense cortex: no need brace, may never fracture.
II	Widened cortex, narrow IM + trabeculation failure: brace. union rate 91%
III	Cystic: pre fracture. Early fixation. Union rate 75%
IV	Dysplastic, fracture, cyst, pseudoarthrosis. Union rate 70%

•Hx, PE

•Age, FHx, associated abnormalities (of NF and tibial hemimelia), previous treatment, LLD, angular deformity, joint stiffness/ OA

General management aim:

•Promote union → Prevent fracture

•Correct deformity + Equal leg length

•Natural history: vicious cycle of limb angulation, repeated #, LLD, joint stiffness

•Check/perform counsel patient’s family from the outset about possibility of amputation

•If prefracture – Clampshell brace, no evidence to suggest bracing will prevent fracture or deformity

•If impending fracture/ fracture / pseudoarthrosis, MRI first to see extent of pseudoarthrosis and how much to resect

•4 in 1 osteosynthesis

•Resection of pseudoarthrosis + fibrotic periosteum

•Correct angular deformity → 3 options: bone grafting, acute shortening, bone transport

•Enhance biology with BMP2,7, periosteal flap, autogenous bone graft (limited source from child) [E: iliac graft]

•Stable fixation fixation by ilizarov + IMN (through ankle joint) [often most difficult part due to small bone → William’s rod]

•Serial observation + bracing until maturity

•EPOS (european paediatric orthopaedic society) 2000 Gill

•340 patients

•Pre fracture clampshell brace

•# → ilizarov (provide compression, also allow lengthening/ bone transport) → IM nail (antegrade rush pin, or splint ankle to control distal fragment and to prevent future #, or William rod – through STJ and TTJ if <4yo, proximalise it if postop 2 years and not grown out of ankle joint yet )

•Poor prognostic factors are 1. young age, 2. advanced Crawford staging, 3. NF (not mentioned: fibular pseudoarthrosis)

•3 unsuccessful OT/ severe LLD (>15cm)/ non functional → amputate (SYMES, prosthesis protect pseudoarthrosis)

Argument for timing of surgery

•For older – difficult to provide stable fixation with any technique in small child, even with ilizarov. Also difficult to ask them to weight bear with the frame, which is needed to stimulate bone growth

•For younger – if delay till older. A braced and protected leg cannot develop normally and will become dysplastic, lose function, become shorter due to growth retardation.

•Therefore, based on EPOS – reasonable success between 3-6 yo (the fibromatosis of the tissue found in the area of pseudoarthrosis has greater osteolytic activity in smaller children)

Janus:

Principle mx:

•Prevent #

•Coz once #, diffi to heal

key point in OT:

•Cross union

•4 in 1 procedure

•Coz patho harmartoma/ periosteaum

•Periosteal graft from iliac crest

•Excise harmartoma, recanalize

•X guided growth – coz deformity is in diaphysis not in physis

•So osteotomy

MRI not necessary if XR definite craford 2

Intraop assessment x firbrous tissue/ harmartoma

**Fig 1.** Clinical photograph - tibial pseudoarthrosis clinical photographtop left

•Functional outcome, at maturity

•FWB 40%

•LLD 60%

•Walk unlimited 70%

•N Ankle 30%

•Sports <30%

Pathology:

•Growth of fibrous harmatoma in pathological periosteum → strangulation of blood supply, decrease osteogenicity, increased osteoclastic resorption

•Dysplastic bone → failure of new bone formation and segmental osseous weakening

•Anterolateral bowing of tibia associated with NF (50%), ED syndrome, amnionic band syndrome

Why is Ilizarov the method of choice?

According to EPOS study, advantage:

•Excellent stability

•Allows complete resection of the pseudoarthritic area regardless of the amount of resected segment because this device allows lengthening or segmental bone transport

•Enables weight bearing , which stimulates healing of bone and soft tissue

•Can also be used to transport the fibula distally, avoid valgus ankle deformity, which is a problem commonly overlooked and underestimated

•Bone transport vs acute shortening + LLD correction via proximal metaphyseal lengthening after corticotomy

•Bone transport lower fusion rate

•Deep muscles and periosteum move with the transported segment but the superficial muscle and fascia move very little

•During transport, major vessels lengthen in the region of regeneration and become tortuous distally, causing kinking of vessels

•→ EPOS study suggest acute shortening + LLD correction via proximal metaphyseal lengthening after corticotomy

#	lvl	marL	indent	logical	original note line
1	0	None	None	0	Clinically photo showing patient’s right leg with abnormal angulation at level of distal tibia, with apex anterior, on frontal view there is no significant angulation (AL bowing), multiple hyperpigmented skin lesions ?scar/ café au lait spots
2	0	None	None	0	Any neurofibroma / axillary freckles
3	0	None	None	0	Anterior bowing of tibia is likely pathological
4	0	0	0	0	Xray showing abnormalities of distal tibia and fibula, # with ?pseudoarthrosis over tibia, fibula length is normal → Dx Tibial pseudoarthrosis
5	0	None	None	0	Ddx:
6	0	171450	-171450	1	NF with tibial pseudoarthrosis
7	0	171450	-171450	1	Tibial hemimelia (anterolateral bowing)
8	0	171450	-171450	1	Fibrous dysplasia
9	0	171450	-171450	1	Trauma with fracture callus
10	0	171450	-171450	1	Tumor (EWS, osteofibrous dysplasia, adamantinoma)
11	0	171450	-171450	1	Osteomyelitis
12	0	0	0	0	Crawford classification. Others: Anderson, Boyd
13	1	None	None	1	I – Dense cortex: no need brace, may never fracture.
14	1	None	None	1	II – Widened cortex, narrow IM + trabeculation failure: brace. union rate 91%
15	1	None	None	1	III – Cystic: pre fracture. Early fixation. Union rate 75%
16	1	None	None	1	IV – Dysplastic, fracture, cyst, pseudoarthrosis. Union rate 70%
17	0	None	None	0	Hx, PE
18	0	None	None	0	Age, FHx, associated abnormalities (of NF and tibial hemimelia), previous treatment, LLD, angular deformity, joint stiffness/ OA
19	0	None	None	0	General management aim:
20	0	228600	-228600	1	Promote union → Prevent fracture
21	0	228600	-228600	1	Correct deformity + Equal leg length
22	0	0	0	0	Natural history: vicious cycle of limb angulation, repeated #, LLD, joint stiffness
23	0	0	0	0	Need to counsel patient’s family from the outset about possibility of amputation
24	0	0	0	0	If prefracture – Clampshell brace, no evidence to suggest bracing will prevent fracture or deformity
25	0	0	0	0	If impending fracture/ fracture / pseudoarthrosis, MRI first to see extent of pseudoarthrosis and how much to resect
26	0	0	0	0	4 in 1 osteosynthesis
27	0	228600	-228600	1	Resection of pseudoarthrosis + fibrotic periosteum
28	0	228600	-228600	1	Correct angular deformity → 3 options: bone grafting, acute shortening, bone transport
29	0	228600	-228600	1	Enhance biology with BMP2,7, periosteal flap, autogenous bone graft (limited source from child) [E: iliac graft]
30	0	228600	-228600	1	Stable fixation fixation by ilizarov + IMN (through ankle joint) [often most difficult part due to small bone --> William’s rod]
31	0	228600	-228600	1	Serial observation + bracing until maturity
32	0	None	None	0	EPOS (european paediatric orthopaedic society) 2000 Gill
33	1	None	None	1	340 patients
34	1	None	None	1	Pre fracture clampshell brace
35	1	None	None	1	# → ilizarov (provide compression, also allow lengthening/ bone transport) → IM nail (antegrade rush pin, or splint ankle to control distal fragment and to prevent future #, or William rod – through STJ and TTJ if <4yo, proximalise it if postop 2 years and not grown out of ankle joint yet )
36	1	None	None	1	Poor prognostic factors are 1. young age, 2. advanced Crawford staging, 3. NF (not mentioned: fibular pseudoarthrosis)
37	0	None	None	0	3 unsuccessful OT/ severe LLD (>15cm)/ non functional → amputate (SYMES, prosthesis protect pseudoarthrosis)
38	0	None	None	0	Argument for timing of surgery
39	0	None	None	0	For older – difficult to provide stable fixation with any technique in small child, even with ilizarov. Also difficult to ask them to weight bear with the frame, which is needed to stimulate bone growth
40	0	None	None	0	For younger – if delay till older. A braced and protected leg cannot develop normally and will become dysplastic, lose function, become shorter due to growth retardation.
41	0	None	None	0	Therefore, based on EPOS – reasonable success between 3-6 yo (the fibromatosis of the tissue found in the area of pseudoarthrosis has greater osteolytic activity in smaller children)
42	0	None	None	0	Janus:
43	0	None	None	0	Principle mx:
44	0	None	None	0	Prevent #
45	0	None	None	0	Coz once #, diffi to heal
46	0	None	None	0	key point in OT:
47	0	None	None	0	Cross union
48	0	None	None	0	4 in 1 procedure
49	0	None	None	0	Coz patho harmartoma/ periosteaum
50	0	None	None	0	Periosteal graft from iliac crest
51	0	None	None	0	Excise harmartoma, recanalize
52	0	None	None	0	X guided growth – coz deformity is in diaphysis not in physis
53	0	None	None	0	So osteotomy
54	0	None	None	0	MRI not necessary if XR definite craford 2
55	0	None	None	0	Intraop assessment x firbrous tissue/ harmartoma
56	0	None	None	0	==============================================
57	0	None	None	0	Functional outcome, at maturity
58	0	None	None	0	FWB 40%
59	0	None	None	0	LLD 60%
60	0	None	None	0	Walk unlimited 70%
61	0	None	None	0	N Ankle 30%
62	0	None	None	0	Sports <30%
63	0	0	0	0	Pathology:
64	0	0	0	0	Growth of fibrous harmatoma in pathological periosteum → strangulation of blood supply, decrease osteogenicity, increased osteoclastic resorption
65	0	0	0	0	Dysplastic bone → failure of new bone formation and segmental osseous weakening
66	0	0	0	0	Anterolateral bowing of tibia associated with NF (50%), ED syndrome, amnionic band syndrome
67	0	0	0	0	Why is Ilizarov the method of choice?
68	0	0	0	0	According to EPOS study, advantage:
69	0	228600	-228600	1	Excellent stability
70	0	228600	-228600	1	Allows complete resection of the pseudoarthritic area regardless of the amount of resected segment because this device allows lengthening or segmental bone transport
71	0	228600	-228600	1	Enables weight bearing , which stimulates healing of bone and soft tissue
72	0	228600	-228600	1	Can also be used to transport the fibula distally, avoid valgus ankle deformity, which is a problem commonly overlooked and underestimated
73	0	0	0	0	Bone transport vs acute shortening + LLD correction via proximal metaphyseal lengthening after corticotomy
74	0	0	0	0	Bone transport lower fusion rate
75	0	171450	-171450	1	Deep muscles and periosteum move with the transported segment but the superficial muscle and fascia move very little
76	0	171450	-171450	1	During transport, major vessels lengthen in the region of regeneration and become tortuous distally, causing kinking of vessels
77	0	0	0	0	→ EPOS study suggest acute shortening + LLD correction via proximal metaphyseal lengthening after corticotomy

#	semantic level	section?	rewritten line
1	0		Clinically photo showing patient’s right leg with abnormal angulation at level of distal tibia, with apex anterior, on frontal view there is no significant angulation (AL bowing), multiple hyperpigmented skin lesions ?scar/ café au lait spots
2	1		Any neurofibroma / axillary freckles
3	1		Anterior bowing of tibia is likely pathological
4	1		Xray showing abnormalities of distal tibia and fibula, # with ?pseudoarthrosis over tibia, fibula length is normal → Dx Tibial pseudoarthrosis
5	0	yes	Differential diagnosis
6	1		NF with tibial pseudoarthrosis
7	1		Tibial hemimelia (anterolateral bowing)
8	1		Fibrous dysplasia
9	1		Trauma with fracture callus
10	1		Tumor (EWS, osteofibrous dysplasia, adamantinoma)
11	1		Osteomyelitis
12	0	yes	Crawford classification. Others: Anderson, Boyd
13	1		I – Dense cortex: no need brace, may never fracture.
14	1		II – Widened cortex, narrow IM + trabeculation failure: brace. union rate 91%
15	1		III – Cystic: pre fracture. Early fixation. Union rate 75%
16	1		IV – Dysplastic, fracture, cyst, pseudoarthrosis. Union rate 70%
17	1		Hx, PE
18	1		Age, FHx, associated abnormalities (of NF and tibial hemimelia), previous treatment, LLD, angular deformity, joint stiffness/ OA
19	0	yes	General management aim:
20	1		Promote union → Prevent fracture
21	1		Correct deformity + Equal leg length
22	1		Natural history: vicious cycle of limb angulation, repeated #, LLD, joint stiffness
23	1		Check/perform counsel patient’s family from the outset about possibility of amputation
24	1		If prefracture – Clampshell brace, no evidence to suggest bracing will prevent fracture or deformity
25	1		If impending fracture/ fracture / pseudoarthrosis, MRI first to see extent of pseudoarthrosis and how much to resect
26	1		4 in 1 osteosynthesis
27	1		Resection of pseudoarthrosis + fibrotic periosteum
28	1		Correct angular deformity → 3 options: bone grafting, acute shortening, bone transport
29	1		Enhance biology with BMP2,7, periosteal flap, autogenous bone graft (limited source from child) [E: iliac graft]
30	1		Stable fixation fixation by ilizarov + IMN (through ankle joint) [often most difficult part due to small bone → William’s rod]
31	1		Serial observation + bracing until maturity
32	1		EPOS (european paediatric orthopaedic society) 2000 Gill
33	1		340 patients
34	1		Pre fracture clampshell brace
35	1		# → ilizarov (provide compression, also allow lengthening/ bone transport) → IM nail (antegrade rush pin, or splint ankle to control distal fragment and to prevent future #, or William rod – through STJ and TTJ if <4yo, proximalise it if postop 2 years and not grown out of ankle joint yet )
36	1		Poor prognostic factors are 1. young age, 2. advanced Crawford staging, 3. NF (not mentioned: fibular pseudoarthrosis)
37	1		3 unsuccessful OT/ severe LLD (>15cm)/ non functional → amputate (SYMES, prosthesis protect pseudoarthrosis)
38	0	yes	Argument for timing of surgery
39	1		For older – difficult to provide stable fixation with any technique in small child, even with ilizarov. Also difficult to ask them to weight bear with the frame, which is needed to stimulate bone growth
40	1		For younger – if delay till older. A braced and protected leg cannot develop normally and will become dysplastic, lose function, become shorter due to growth retardation.
41	1		Therefore, based on EPOS – reasonable success between 3-6 yo (the fibromatosis of the tissue found in the area of pseudoarthrosis has greater osteolytic activity in smaller children)
42	0	yes	Janus:
43	0	yes	Principle mx:
44	1		Prevent #
45	1		Coz once #, diffi to heal
46	0	yes	key point in OT:
47	1		Cross union
48	1		4 in 1 procedure
49	1		Coz patho harmartoma/ periosteaum
50	1		Periosteal graft from iliac crest
51	1		Excise harmartoma, recanalize
52	1		X guided growth – coz deformity is in diaphysis not in physis
53	1		So osteotomy
54	0	yes	MRI not necessary if XR definite craford 2
55	0	yes	Intraop assessment x firbrous tissue/ harmartoma
56	0		——
57	1		Functional outcome, at maturity
58	1		FWB 40%
59	1		LLD 60%
60	1		Walk unlimited 70%
61	1		N Ankle 30%
62	1		Sports <30%
63	0	yes	Pathology:
64	1		Growth of fibrous harmatoma in pathological periosteum → strangulation of blood supply, decrease osteogenicity, increased osteoclastic resorption
65	1		Dysplastic bone → failure of new bone formation and segmental osseous weakening
66	1		Anterolateral bowing of tibia associated with NF (50%), ED syndrome, amnionic band syndrome
67	0	yes	Why is Ilizarov the method of choice?
68	0	yes	According to EPOS study, advantage:
69	1		Excellent stability
70	1		Allows complete resection of the pseudoarthritic area regardless of the amount of resected segment because this device allows lengthening or segmental bone transport
71	1		Enables weight bearing , which stimulates healing of bone and soft tissue
72	1		Can also be used to transport the fibula distally, avoid valgus ankle deformity, which is a problem commonly overlooked and underestimated
73	1		Bone transport vs acute shortening + LLD correction via proximal metaphyseal lengthening after corticotomy
74	1		Bone transport lower fusion rate
75	1		Deep muscles and periosteum move with the transported segment but the superficial muscle and fascia move very little
76	1		During transport, major vessels lengthen in the region of regeneration and become tortuous distally, causing kinking of vessels
77	1		→ EPOS study suggest acute shortening + LLD correction via proximal metaphyseal lengthening after corticotomy

024. Congenital tibial pseudoarthrosis / anterolateral tibial bowing

Clean FRCS notes with inline figures

Differential diagnosis

Crawford classification. Others: Anderson, Boyd

General management aim:

Argument for timing of surgery

Janus:

Principle mx:

key point in OT:

MRI not necessary if XR definite craford 2

Intraop assessment x firbrous tissue/ harmartoma

Pathology:

Why is Ilizarov the method of choice?

According to EPOS study, advantage:

Direct Q&A

NF with tibial pseudoarthrosis

Promote union → Prevent fracture

Natural history: vicious cycle of limb angulation, repeated #, LLD, joint stiffness

Functional outcome, at maturity

Excellent stability

Raw speaker-note hierarchy audit

Improved semantic hierarchy used for the study notes