Slide 066 — MHE

Clean FRCS notes with inline figures

MHE

•XR of both knees showing a skeletally mature patient with multiple bony protrusions of the metaphyseal region of distal femur and proximal tibia with associated tubulation of the metaphyseal region.

•The protrusions are pedunculated, well defined, with a narrowing zone of transition and pointing away from the physis. Trabeculae are seen within the lesion and are continuous with the rest of the bone.

•Otherwise there are no cortical destruction, no calcification within the soft tissue indicating a malignant transformation.

•Dx Multiple hereditary exostosis

•Ddx: Trevor disease (dysplasia epiphysealis hemimelia): Epiphyseal exostosis

How to differentiate?

•Clinically: Trevor younger

•Radiological: Trevor arise from epiphysis

•Histological: cartilage cap of Trevor contain bands of cartilage separating areas of cancellous bone

•Gene: normal EXT levels

•Also check clinically the forearms to see if there are any bony exostosis and associated radial head dislocation and bowing

Investigations

•Further XR to look at the scapular and pelvis (regions where clinically cannot be palpated)

•Scanogram for LL alignment

Management

•Benign condition

Rule out complication

•Local pressure sx (nerve, tendon, bursitis, pseudoaneurysm)

•Reduce joint ROM

•Deformity / growth disturbance

•Malignancy

•Stop growing at maturity

•Monitor → 5-10% lifetime risk of malignant transformation (c/w normal lifetime risk of any malignancy of 1 in 3)

Indication for surgery

•Pain from bursitis / affect joint movement / compression on NV and tendon

•Deformity (bowing of forearm, genu varum and valgum), intraarticular exostosis

•Malignant transformation into chondrosarcoma (@ 5th decade) 1% lifetime risk per lesion, thickness >2cm (normal 2-3mm)

•Clinical: Increase in size after puberty, acute onset of pain in adults

•Radiological: cortical destruction, soft tissue mineralization >2cm, MRI cartilage cap >2cm, sessile, central more malignancy

Principle of surgery:

•Excise lesion including cartilaginous cap to reduce recurrence

•Corrective osteotomy +/- lengthening procedures

•MHE is a benign condition with multiple chondrogenic tumor (Defect in the groove of Ranvier, aberrant cartilage from the perichondral ring, mutations affecting prehypertrophic chondrocytes)

•Sessile / pedunculated growth from physeal plate away from joint

•AD, EXT1 exostosin (Chr 8), 2 (Chr 11), 3 gene (Chr 19) (1 is worst, more exostosis, more sarcoma, more limb deformities, more axial bone involved)

•10% spontaneous mutation

Rubin classification: metaphyseal hyperplasia

Direct Q&A

Q1 How to differentiate?

Clinically: Trevor younger

•Radiological: Trevor arise from epiphysis

•Histological: cartilage cap of Trevor contain bands of cartilage separating areas of cancellous bone

•Gene: normal EXT levels

•Also check clinically the forearms to see if there are any bony exostosis and associated radial head dislocation and bowing

Q2 What investigations are listed for MHE?

•Further XR to look at the scapular and pelvis (regions where clinically cannot be palpated)

•Scanogram for LL alignment

Q3 How is MHE managed?

•Benign condition

Q4 What complications are listed for MHE?

Local pressure sx (nerve, tendon, bursitis, pseudoaneurysm)

•Reduce joint ROM

•Deformity / growth disturbance

•Malignancy

•Stop growing at maturity

•Monitor → 5-10% lifetime risk of malignant transformation (c/w normal lifetime risk of any malignancy of 1 in 3)

Q5 What operative steps, indications, and risks are listed for Indication for surgery?

•Pain from bursitis / affect joint movement / compression on NV and tendon

•Deformity (bowing of forearm, genu varum and valgum), intraarticular exostosis

•Malignant transformation into chondrosarcoma (@ 5th decade) 1% lifetime risk per lesion, thickness >2cm (normal 2-3mm)

•Clinical: Increase in size after puberty, acute onset of pain in adults

•Radiological: cortical destruction, soft tissue mineralization >2cm, MRI cartilage cap >2cm, sessile, central more malignancy

Q6 What operative steps, indications, and risks are listed for Principle of surgery?

Excise lesion including cartilaginous cap to reduce recurrence

•Corrective osteotomy +/- lengthening procedures

Raw speaker-note hierarchy audit

#	lvl	marL	indent	logical	original note line
1	0	None	None	0	MHE
2	0	None	None	0	XR of both knees showing a skeletally mature patient with multiple bony protrusions of the metaphyseal region of distal femur and proximal tibia with associated tubulation of the metaphyseal region.
3	0	None	None	0	The protrusions are pedunculated, well defined, with a narrowing zone of transition and pointing away from the physis. Trabeculae are seen within the lesion and are continuous with the rest of the bone.
4	0	None	None	0	Otherwise there are no cortical destruction, no calcification within the soft tissue indicating a malignant transformation.
5	0	None	None	0	Dx Multiple hereditary exostosis
6	0	0	0	0	Ddx: Trevor disease (dysplasia epiphysealis hemimelia): Epiphyseal exostosis
7	0	0	0	0	How to differentiate?
8	0	0	0	0	Clinically: Trevor younger
9	0	0	0	0	Radiological: Trevor arise from epiphysis
10	0	0	0	0	Histological: cartilage cap of Trevor contain bands of cartilage separating areas of cancellous bone
11	0	0	0	0	Gene: normal EXT levels
12	0	0	0	0	Also check clinically the forearms to see if there are any bony exostosis and associated radial head dislocation and bowing
13	0	0	0	0	Ix:
14	0	0	0	0	Further XR to look at the scapular and pelvis (regions where clinically cannot be palpated)
15	0	0	0	0	Scanogram for LL alignment
16	0	None	None	0	Mx:
17	0	None	None	0	Benign condition
18	0	None	None	0	Rule out complication
19	0	171450	-171450	1	Local pressure sx (nerve, tendon, bursitis, pseudoaneurysm)
20	0	171450	-171450	1	Reduce joint ROM
21	0	171450	-171450	1	Deformity / growth disturbance
22	0	171450	-171450	1	Malignancy
23	0	None	None	0	Stop growing at maturity
24	0	None	None	0	Monitor → 5-10% lifetime risk of malignant transformation (c/w normal lifetime risk of any malignancy of 1 in 3)
25	0	None	None	0	Indication for surgery
26	1	971550	-514350	3	Pain from bursitis / affect joint movement / compression on NV and tendon
27	1	971550	-514350	3	Deformity (bowing of forearm, genu varum and valgum), intraarticular exostosis
28	1	971550	-514350	3	Malignant transformation into chondrosarcoma (@ 5th decade) 1% lifetime risk per lesion, thickness >2cm (normal 2-3mm)
29	0	None	None	0	Malignancy?
30	0	228600	-228600	1	Clinical: Increase in size after puberty, acute onset of pain in adults
31	0	228600	-228600	1	Radiological: cortical destruction, soft tissue mineralization >2cm, MRI cartilage cap >2cm, sessile, central more malignancy
32	0	None	None	0	Principle of surgery:
33	0	171450	-171450	1	Excise lesion including cartilaginous cap to reduce recurrence
34	0	171450	-171450	1	Corrective osteotomy +/- lengthening procedures
35	0	0	0	0	=====================================
36	0	None	None	0	MHE is a benign condition with multiple chondrogenic tumor (Defect in the groove of Ranvier, aberrant cartilage from the perichondral ring, mutations affecting prehypertrophic chondrocytes)
37	0	None	None	0	Sessile / pedunculated growth from physeal plate away from joint
38	0	None	None	0	AD, EXT1 exostosin (Chr 8), 2 (Chr 11), 3 gene (Chr 19) (1 is worst, more exostosis, more sarcoma, more limb deformities, more axial bone involved)
39	0	None	None	0	10% spontaneous mutation
40	0	None	None	0	Rubin classification: metaphyseal hyperplasia

lvl

marL

indent

logical

original note line

None

MHE

None

XR of both knees showing a skeletally mature patient with multiple bony protrusions of the metaphyseal region of distal femur and proximal tibia with associated tubulation of the metaphyseal region.

None

The protrusions are pedunculated, well defined, with a narrowing zone of transition and pointing away from the physis. Trabeculae are seen within the lesion and are continuous with the rest of the bone.

None

Otherwise there are no cortical destruction, no calcification within the soft tissue indicating a malignant transformation.

None

Dx Multiple hereditary exostosis

Ddx: Trevor disease (dysplasia epiphysealis hemimelia): Epiphyseal exostosis

How to differentiate?

Clinically: Trevor younger

Radiological: Trevor arise from epiphysis

Histological: cartilage cap of Trevor contain bands of cartilage separating areas of cancellous bone

Gene: normal EXT levels

Also check clinically the forearms to see if there are any bony exostosis and associated radial head dislocation and bowing

Ix:

Further XR to look at the scapular and pelvis (regions where clinically cannot be palpated)

Scanogram for LL alignment

None

Mx:

None

Benign condition

None

Rule out complication

171450

-171450

Local pressure sx (nerve, tendon, bursitis, pseudoaneurysm)

171450

-171450

Reduce joint ROM

171450

-171450

Deformity / growth disturbance

171450

-171450

Malignancy

None

Stop growing at maturity

None

Monitor → 5-10% lifetime risk of malignant transformation (c/w normal lifetime risk of any malignancy of 1 in 3)

None

Indication for surgery

971550

-514350

Pain from bursitis / affect joint movement / compression on NV and tendon

971550

-514350

Deformity (bowing of forearm, genu varum and valgum), intraarticular exostosis

971550

-514350

Malignant transformation into chondrosarcoma (@ 5th decade) 1% lifetime risk per lesion, thickness >2cm (normal 2-3mm)

None

Malignancy?

228600

-228600

Clinical: Increase in size after puberty, acute onset of pain in adults

228600

-228600

Radiological: cortical destruction, soft tissue mineralization >2cm, MRI cartilage cap >2cm, sessile, central more malignancy

None

Principle of surgery:

171450

-171450

Excise lesion including cartilaginous cap to reduce recurrence

171450

-171450

Corrective osteotomy +/- lengthening procedures

=====================================

None

MHE is a benign condition with multiple chondrogenic tumor (Defect in the groove of Ranvier, aberrant cartilage from the perichondral ring, mutations affecting prehypertrophic chondrocytes)

None

Sessile / pedunculated growth from physeal plate away from joint

None

AD, EXT1 exostosin (Chr 8), 2 (Chr 11), 3 gene (Chr 19) (1 is worst, more exostosis, more sarcoma, more limb deformities, more axial bone involved)

None

10% spontaneous mutation

None

Rubin classification: metaphyseal hyperplasia

Improved semantic hierarchy used for the study notes

#	semantic level	section?	rewritten line
1	0		MHE
2	1		XR of both knees showing a skeletally mature patient with multiple bony protrusions of the metaphyseal region of distal femur and proximal tibia with associated tubulation of the metaphyseal region.
3	1		The protrusions are pedunculated, well defined, with a narrowing zone of transition and pointing away from the physis. Trabeculae are seen within the lesion and are continuous with the rest of the bone.
4	1		Otherwise there are no cortical destruction, no calcification within the soft tissue indicating a malignant transformation.
5	1		Dx Multiple hereditary exostosis
6	1		Ddx: Trevor disease (dysplasia epiphysealis hemimelia): Epiphyseal exostosis
7	0	yes	How to differentiate?
8	1		Clinically: Trevor younger
9	1		Radiological: Trevor arise from epiphysis
10	1		Histological: cartilage cap of Trevor contain bands of cartilage separating areas of cancellous bone
11	1		Gene: normal EXT levels
12	1		Also check clinically the forearms to see if there are any bony exostosis and associated radial head dislocation and bowing
13	0	yes	Investigations
14	1		Further XR to look at the scapular and pelvis (regions where clinically cannot be palpated)
15	1		Scanogram for LL alignment
16	0	yes	Management
17	1		Benign condition
18	0	yes	Rule out complication
19	1		Local pressure sx (nerve, tendon, bursitis, pseudoaneurysm)
20	1		Reduce joint ROM
21	1		Deformity / growth disturbance
22	1		Malignancy
23	1		Stop growing at maturity
24	1		Monitor → 5-10% lifetime risk of malignant transformation (c/w normal lifetime risk of any malignancy of 1 in 3)
25	0	yes	Indication for surgery
26	3		Pain from bursitis / affect joint movement / compression on NV and tendon
27	3		Deformity (bowing of forearm, genu varum and valgum), intraarticular exostosis
28	3		Malignant transformation into chondrosarcoma (@ 5th decade) 1% lifetime risk per lesion, thickness >2cm (normal 2-3mm)
29	1		Clinical: Increase in size after puberty, acute onset of pain in adults
30	1		Radiological: cortical destruction, soft tissue mineralization >2cm, MRI cartilage cap >2cm, sessile, central more malignancy
31	0	yes	Principle of surgery:
32	1		Excise lesion including cartilaginous cap to reduce recurrence
33	1		Corrective osteotomy +/- lengthening procedures
34	0		——
35	1		MHE is a benign condition with multiple chondrogenic tumor (Defect in the groove of Ranvier, aberrant cartilage from the perichondral ring, mutations affecting prehypertrophic chondrocytes)
36	1		Sessile / pedunculated growth from physeal plate away from joint
37	1		AD, EXT1 exostosin (Chr 8), 2 (Chr 11), 3 gene (Chr 19) (1 is worst, more exostosis, more sarcoma, more limb deformities, more axial bone involved)
38	1		10% spontaneous mutation
39	0	yes	Rubin classification: metaphyseal hyperplasia

semantic level

section?

rewritten line

MHE

XR of both knees showing a skeletally mature patient with multiple bony protrusions of the metaphyseal region of distal femur and proximal tibia with associated tubulation of the metaphyseal region.

Otherwise there are no cortical destruction, no calcification within the soft tissue indicating a malignant transformation.

Dx Multiple hereditary exostosis

Ddx: Trevor disease (dysplasia epiphysealis hemimelia): Epiphyseal exostosis

yes

How to differentiate?

Clinically: Trevor younger

Radiological: Trevor arise from epiphysis

Histological: cartilage cap of Trevor contain bands of cartilage separating areas of cancellous bone

Gene: normal EXT levels

Also check clinically the forearms to see if there are any bony exostosis and associated radial head dislocation and bowing

yes

Investigations

Further XR to look at the scapular and pelvis (regions where clinically cannot be palpated)

Scanogram for LL alignment

yes

Management

Benign condition

yes

Rule out complication

Local pressure sx (nerve, tendon, bursitis, pseudoaneurysm)

Reduce joint ROM

Deformity / growth disturbance

Malignancy

Stop growing at maturity

Monitor → 5-10% lifetime risk of malignant transformation (c/w normal lifetime risk of any malignancy of 1 in 3)

yes

Indication for surgery

Pain from bursitis / affect joint movement / compression on NV and tendon

Deformity (bowing of forearm, genu varum and valgum), intraarticular exostosis

Malignant transformation into chondrosarcoma (@ 5th decade) 1% lifetime risk per lesion, thickness >2cm (normal 2-3mm)

Clinical: Increase in size after puberty, acute onset of pain in adults

Radiological: cortical destruction, soft tissue mineralization >2cm, MRI cartilage cap >2cm, sessile, central more malignancy

yes

Principle of surgery:

Excise lesion including cartilaginous cap to reduce recurrence

Corrective osteotomy +/- lengthening procedures

——

MHE is a benign condition with multiple chondrogenic tumor (Defect in the groove of Ranvier, aberrant cartilage from the perichondral ring, mutations affecting prehypertrophic chondrocytes)

Sessile / pedunculated growth from physeal plate away from joint

AD, EXT1 exostosin (Chr 8), 2 (Chr 11), 3 gene (Chr 19) (1 is worst, more exostosis, more sarcoma, more limb deformities, more axial bone involved)

10% spontaneous mutation

yes

Rubin classification: metaphyseal hyperplasia

066. MHE

Clean FRCS notes with inline figures

MHE

How to differentiate?

Investigations

Management

Rule out complication

Indication for surgery

Principle of surgery:

Rubin classification: metaphyseal hyperplasia

Direct Q&A

Clinically: Trevor younger

Local pressure sx (nerve, tendon, bursitis, pseudoaneurysm)

Excise lesion including cartilaginous cap to reduce recurrence

Raw speaker-note hierarchy audit

Improved semantic hierarchy used for the study notes