Slide 070 — Sprengel shoulder

Clean FRCS notes with inline figures

Clinical photo showing patient with asymmetrical scapula

•Right sided scapula is hypoplastic and undescended

•Hairline also appears low

•Short webbed neck

•No scapular winging

•Also look from the front to see if there is upward tilting of clavicle

•This is a Sprengel shoulder

Associations:

•1. Klippel Feil syndrome

•2. Congenital scoliosis

•3. Cervical spinal bifida

•4. VACTERL +/- situ invertus

Examination

•Neck: Short, stiff

•Document omovertebral bar, shoulder movement, abduction power (medial wing + superomedial angle move superiorly → glenoid pointed inferiorly)

•Shoulder abduction: movement only coming from GHJ, no STJ movement

•Look for UL abnormalities (VACTERL), periscapular muscle fibrosis / weakened, check distal NV status (syringomyelia)

•Look for scoliosis/ kyphosis/ back pitting (spina bifida)

Xray

•Omovertebral bar (bar best seen on lateral/ oblique Xray of C spine)

•Clavicle uptilt

•High riding scapula

•Small scapula (Transverse > vertical)

•Inferomedial corner medial tilt + glenoid downward tilt

•Association with Klippel Feil: C spine failure of segmentation, C1/2 instability, Cranial settling

•Need CT and MRI spine to look for bar and spinal problem such as dural ectasia or diastematomyelia

•Also cardiac and renal workup, also rule out other C spine problems

Staging:

•Caverndish

•Very mild, not detectable in clothing

•Mild 1-2cm higher

•Moderate 2-5cm higher

•Severe >5cm higher (superior angle near occiput)

Management

•OT if 1. Cosmetic problem, 2. Abduction < 120 @ 3-8yo (too late more nerve injury)

Aims:

•1. Excise omovertebral bar + superior trapezius release at C4

•2. Relocation of scapula

•Woodward (detachment and distal advancement of the midline origin of parascapular muscles)

•Green (extraperiosteal detachment of periscapular muscle, then hold the scapula in its new position with spring wire traction and spica casting for 3 weeks)

•Both improve 50deg

•3. Clavicle morselized osteotomy → preserve periosteum (decrease brachial plexus injury)

•→ Principle is to improve shoulder abduction (will improve 30 deg, Caverndish by 1 grade)

•Congenital undescended scapula

•From 4-6th C spine (pharyngeal arch), caudal migration to T2-7 during 9th to 12th weeks of gestation

Interruption of embryonic subclavian blood supply

•10-30% bilateral

•Present in 1/3 Klippel-Feil

•Association: scoliosis, spinal dysraphism, VACTERL

•Klippel Feil Syndrome (SGM 1 gene at chromosome 8)

•Congenital condition with fusion of more than two cervical vertebra

Classical Triad

•Low posterior hair line

•Short webbed neck

•Decreased cervical movement (normal F/E, decrease side bending)

•(Hearing loss, micrognathia, torticollis, scoliosis)

Xray triad

•Unsegmented cervical spine (commonest C2/3)

•ADI, C1/2 instability

•Adjacent segment degeneration

•(may also have basilar invagination)

•If involve C2 → no contact sports

•If lower → usually asymptomatic, until adjacent levels degenerate (100%)→ contact sports ok

•OT if 1. pain, 2. instability, 3. neurology

•Check/perform check CVS system and urogenital system before OT

Direct Q&A

Q1 What examination findings should be assessed in Sprengel shoulder?

Neck: Short, stiff

•Document omovertebral bar, shoulder movement, abduction power (medial wing + superomedial angle move superiorly → glenoid pointed inferiorly)

•Shoulder abduction: movement only coming from GHJ, no STJ movement

•Look for UL abnormalities (VACTERL), periscapular muscle fibrosis / weakened, check distal NV status (syringomyelia)

•Look for scoliosis/ kyphosis/ back pitting (spina bifida)

Q2 What are the radiographic findings for Sprengel shoulder?

•Omovertebral bar (bar best seen on lateral/ oblique Xray of C spine)

•Clavicle uptilt

•High riding scapula

•Small scapula (Transverse > vertical)

•Inferomedial corner medial tilt + glenoid downward tilt

•Association with Klippel Feil: C spine failure of segmentation, C1/2 instability, Cranial settling

•Need CT and MRI spine to look for bar and spinal problem such as dural ectasia or diastematomyelia

•Also cardiac and renal workup, also rule out other C spine problems

Q3 How is Sprengel shoulder managed?

•OT if 1. Cosmetic problem, 2. Abduction < 120 @ 3-8yo (too late more nerve injury)

Q4 What are the management aims for Sprengel shoulder?

•1. Excise omovertebral bar + superior trapezius release at C4

•2. Relocation of scapula

•Woodward (detachment and distal advancement of the midline origin of parascapular muscles)

•Green (extraperiosteal detachment of periscapular muscle, then hold the scapula in its new position with spring wire traction and spica casting for 3 weeks)

•Both improve 50deg

•3. Clavicle morselized osteotomy → preserve periosteum (decrease brachial plexus injury)

•→ Principle is to improve shoulder abduction (will improve 30 deg, Caverndish by 1 grade)

Q5 What blood supply pattern is described for Sprengel shoulder?

•10-30% bilateral

•Present in 1/3 Klippel-Feil

•Association: scoliosis, spinal dysraphism, VACTERL

•Klippel Feil Syndrome (SGM 1 gene at chromosome 8)

•Congenital condition with fusion of more than two cervical vertebra

Raw speaker-note hierarchy audit

#	lvl	marL	indent	logical	original note line
1	0	None	None	0	Clinical photo showing patient with asymmetrical scapula
2	0	None	None	0	Right sided scapula is hypoplastic and undescended
3	0	None	None	0	Hairline also appears low
4	0	None	None	0	Short webbed neck
5	0	None	None	0	No scapular winging
6	0	None	None	0	Also look from the front to see if there is upward tilting of clavicle
7	0	None	None	0	This is a Sprengel shoulder
8	0	None	None	0	Associations:
9	1	None	None	1	1. Klippel Feil syndrome
10	1	None	None	1	2. Congenital scoliosis
11	1	None	None	1	3. Cervical spinal bifida
12	1	None	None	1	4. VACTERL +/- situ invertus
13	0	None	None	0	PE
14	0	None	None	0	Neck: Short, stiff
15	0	None	None	0	Document omovertebral bar, shoulder movement, abduction power (medial wing + superomedial angle move superiorly → glenoid pointed inferiorly)
16	0	None	None	0	Shoulder abduction: movement only coming from GHJ, no STJ movement
17	0	None	None	0	Look for UL abnormalities (VACTERL), periscapular muscle fibrosis / weakened, check distal NV status (syringomyelia)
18	0	None	None	0	Look for scoliosis/ kyphosis/ back pitting (spina bifida)
19	0	None	None	0	Xray
20	0	228600	-228600	1	Omovertebral bar (bar best seen on lateral/ oblique Xray of C spine)
21	0	228600	-228600	1	Clavicle uptilt
22	0	228600	-228600	1	High riding scapula
23	0	228600	-228600	1	Small scapula (Transverse > vertical)
24	0	228600	-228600	1	Inferomedial corner medial tilt + glenoid downward tilt
25	0	228600	-228600	1	Association with Klippel Feil: C spine failure of segmentation, C1/2 instability, Cranial settling
26	0	None	None	0	Need CT and MRI spine to look for bar and spinal problem such as dural ectasia or diastematomyelia
27	0	None	None	0	Also cardiac and renal workup, also rule out other C spine problems
28	0	None	None	0	Staging:
29	0	None	None	0	Caverndish
30	1	685800	-228600	2	Very mild, not detectable in clothing
31	1	685800	-228600	2	Mild 1-2cm higher
32	1	685800	-228600	2	Moderate 2-5cm higher
33	1	685800	-228600	2	Severe >5cm higher (superior angle near occiput)
34	0	0	0	0	Mx
35	0	None	None	0	OT if 1. Cosmetic problem, 2. Abduction < 120 @ 3-8yo (too late more nerve injury)
36	0	None	None	0	Aims:
37	0	0	0	0	1. Excise omovertebral bar + superior trapezius release at C4
38	0	0	0	0	2. Relocation of scapula
39	0	171450	-171450	1	Woodward (detachment and distal advancement of the midline origin of parascapular muscles)
40	0	171450	-171450	1	Green (extraperiosteal detachment of periscapular muscle, then hold the scapula in its new position with spring wire traction and spica casting for 3 weeks)
41	0	171450	-171450	1	Both improve 50deg
42	0	None	None	0	3. Clavicle morselized osteotomy → preserve periosteum (decrease brachial plexus injury)
43	0	None	None	0	→ Principle is to improve shoulder abduction (will improve 30 deg, Caverndish by 1 grade)
44	0	None	None	0	==================================================================================
45	0	None	None	0	Congenital undescended scapula
46	0	None	None	0	From 4-6th C spine (pharyngeal arch), caudal migration to T2-7 during 9th to 12th weeks of gestation
47	0	None	None	0	Interruption of embryonic subclavian blood supply
48	0	None	None	0	10-30% bilateral
49	0	None	None	0	Present in 1/3 Klippel-Feil
50	0	None	None	0	Association: scoliosis, spinal dysraphism, VACTERL
51	0	None	None	0	Klippel Feil Syndrome (SGM 1 gene at chromosome 8)
52	0	None	None	0	Congenital condition with fusion of more than two cervical vertebra
53	0	None	None	0	Classical Triad
54	1	685800	-228600	2	Low posterior hair line
55	1	685800	-228600	2	Short webbed neck
56	1	685800	-228600	2	Decreased cervical movement (normal F/E, decrease side bending)
57	1	None	None	1	(Hearing loss, micrognathia, torticollis, scoliosis)
58	0	None	None	0	Xray triad
59	1	685800	-228600	2	Unsegmented cervical spine (commonest C2/3)
60	1	685800	-228600	2	ADI, C1/2 instability
61	1	685800	-228600	2	Adjacent segment degeneration
62	1	None	None	1	(may also have basilar invagination)
63	0	0	0	0	If involve C2 → no contact sports
64	0	0	0	0	If lower → usually asymptomatic, until adjacent levels degenerate (100%)→ contact sports ok
65	0	0	0	0	OT if 1. pain, 2. instability, 3. neurology
66	0	0	0	0	Need to check CVS system and urogenital system before OT

lvl

marL

indent

logical

original note line

None

Clinical photo showing patient with asymmetrical scapula

None

Right sided scapula is hypoplastic and undescended

None

Hairline also appears low

None

Short webbed neck

None

No scapular winging

None

Also look from the front to see if there is upward tilting of clavicle

None

This is a Sprengel shoulder

None

Associations:

None

1. Klippel Feil syndrome

None

2. Congenital scoliosis

None

3. Cervical spinal bifida

None

4. VACTERL +/- situ invertus

None

Neck: Short, stiff

None

Document omovertebral bar, shoulder movement, abduction power (medial wing + superomedial angle move superiorly → glenoid pointed inferiorly)

None

Shoulder abduction: movement only coming from GHJ, no STJ movement

None

Look for UL abnormalities (VACTERL), periscapular muscle fibrosis / weakened, check distal NV status (syringomyelia)

None

Look for scoliosis/ kyphosis/ back pitting (spina bifida)

None

Xray

228600

-228600

Omovertebral bar (bar best seen on lateral/ oblique Xray of C spine)

228600

-228600

Clavicle uptilt

228600

-228600

High riding scapula

228600

-228600

Small scapula (Transverse > vertical)

228600

-228600

Inferomedial corner medial tilt + glenoid downward tilt

228600

-228600

Association with Klippel Feil: C spine failure of segmentation, C1/2 instability, Cranial settling

None

Need CT and MRI spine to look for bar and spinal problem such as dural ectasia or diastematomyelia

None

Also cardiac and renal workup, also rule out other C spine problems

None

Staging:

None

Caverndish

685800

-228600

Very mild, not detectable in clothing

685800

-228600

Mild 1-2cm higher

685800

-228600

Moderate 2-5cm higher

685800

-228600

Severe >5cm higher (superior angle near occiput)

None

OT if 1. Cosmetic problem, 2. Abduction < 120 @ 3-8yo (too late more nerve injury)

None

Aims:

1. Excise omovertebral bar + superior trapezius release at C4

2. Relocation of scapula

171450

-171450

Woodward (detachment and distal advancement of the midline origin of parascapular muscles)

171450

-171450

Green (extraperiosteal detachment of periscapular muscle, then hold the scapula in its new position with spring wire traction and spica casting for 3 weeks)

171450

-171450

Both improve 50deg

None

3. Clavicle morselized osteotomy → preserve periosteum (decrease brachial plexus injury)

None

→ Principle is to improve shoulder abduction (will improve 30 deg, Caverndish by 1 grade)

None

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

None

Congenital undescended scapula

None

From 4-6th C spine (pharyngeal arch), caudal migration to T2-7 during 9th to 12th weeks of gestation

None

Interruption of embryonic subclavian blood supply

None

10-30% bilateral

None

Present in 1/3 Klippel-Feil

None

Association: scoliosis, spinal dysraphism, VACTERL

None

Klippel Feil Syndrome (SGM 1 gene at chromosome 8)

None

Congenital condition with fusion of more than two cervical vertebra

None

Classical Triad

685800

-228600

Low posterior hair line

685800

-228600

Short webbed neck

685800

-228600

Decreased cervical movement (normal F/E, decrease side bending)

None

(Hearing loss, micrognathia, torticollis, scoliosis)

None

Xray triad

685800

-228600

Unsegmented cervical spine (commonest C2/3)

685800

-228600

ADI, C1/2 instability

685800

-228600

Adjacent segment degeneration

None

(may also have basilar invagination)

If involve C2 → no contact sports

If lower → usually asymptomatic, until adjacent levels degenerate (100%)→ contact sports ok

OT if 1. pain, 2. instability, 3. neurology

Need to check CVS system and urogenital system before OT

Improved semantic hierarchy used for the study notes

#	semantic level	section?	rewritten line
1	0		Clinical photo showing patient with asymmetrical scapula
2	1		Right sided scapula is hypoplastic and undescended
3	1		Hairline also appears low
4	1		Short webbed neck
5	1		No scapular winging
6	1		Also look from the front to see if there is upward tilting of clavicle
7	1		This is a Sprengel shoulder
8	0	yes	Associations:
9	1		1. Klippel Feil syndrome
10	1		2. Congenital scoliosis
11	1		3. Cervical spinal bifida
12	1		4. VACTERL +/- situ invertus
13	0	yes	Examination
14	1		Neck: Short, stiff
15	1		Document omovertebral bar, shoulder movement, abduction power (medial wing + superomedial angle move superiorly → glenoid pointed inferiorly)
16	1		Shoulder abduction: movement only coming from GHJ, no STJ movement
17	1		Look for UL abnormalities (VACTERL), periscapular muscle fibrosis / weakened, check distal NV status (syringomyelia)
18	1		Look for scoliosis/ kyphosis/ back pitting (spina bifida)
19	0	yes	Xray
20	1		Omovertebral bar (bar best seen on lateral/ oblique Xray of C spine)
21	1		Clavicle uptilt
22	1		High riding scapula
23	1		Small scapula (Transverse > vertical)
24	1		Inferomedial corner medial tilt + glenoid downward tilt
25	1		Association with Klippel Feil: C spine failure of segmentation, C1/2 instability, Cranial settling
26	1		Need CT and MRI spine to look for bar and spinal problem such as dural ectasia or diastematomyelia
27	1		Also cardiac and renal workup, also rule out other C spine problems
28	0	yes	Staging:
29	1		Caverndish
30	2		Very mild, not detectable in clothing
31	2		Mild 1-2cm higher
32	2		Moderate 2-5cm higher
33	2		Severe >5cm higher (superior angle near occiput)
34	0	yes	Management
35	1		OT if 1. Cosmetic problem, 2. Abduction < 120 @ 3-8yo (too late more nerve injury)
36	0	yes	Aims:
37	1		1. Excise omovertebral bar + superior trapezius release at C4
38	1		2. Relocation of scapula
39	1		Woodward (detachment and distal advancement of the midline origin of parascapular muscles)
40	1		Green (extraperiosteal detachment of periscapular muscle, then hold the scapula in its new position with spring wire traction and spica casting for 3 weeks)
41	1		Both improve 50deg
42	1		3. Clavicle morselized osteotomy → preserve periosteum (decrease brachial plexus injury)
43	1		→ Principle is to improve shoulder abduction (will improve 30 deg, Caverndish by 1 grade)
44	0		——
45	1		Congenital undescended scapula
46	1		From 4-6th C spine (pharyngeal arch), caudal migration to T2-7 during 9th to 12th weeks of gestation
47	0	yes	Interruption of embryonic subclavian blood supply
48	1		10-30% bilateral
49	1		Present in 1/3 Klippel-Feil
50	1		Association: scoliosis, spinal dysraphism, VACTERL
51	1		Klippel Feil Syndrome (SGM 1 gene at chromosome 8)
52	1		Congenital condition with fusion of more than two cervical vertebra
53	0	yes	Classical Triad
54	2		Low posterior hair line
55	2		Short webbed neck
56	2		Decreased cervical movement (normal F/E, decrease side bending)
57	1		(Hearing loss, micrognathia, torticollis, scoliosis)
58	0	yes	Xray triad
59	2		Unsegmented cervical spine (commonest C2/3)
60	2		ADI, C1/2 instability
61	2		Adjacent segment degeneration
62	1		(may also have basilar invagination)
63	1		If involve C2 → no contact sports
64	1		If lower → usually asymptomatic, until adjacent levels degenerate (100%)→ contact sports ok
65	1		OT if 1. pain, 2. instability, 3. neurology
66	1		Check/perform check CVS system and urogenital system before OT

semantic level

section?

rewritten line

Clinical photo showing patient with asymmetrical scapula

Right sided scapula is hypoplastic and undescended

Hairline also appears low

Short webbed neck

No scapular winging

Also look from the front to see if there is upward tilting of clavicle

This is a Sprengel shoulder

yes

Associations:

1. Klippel Feil syndrome

2. Congenital scoliosis

3. Cervical spinal bifida

4. VACTERL +/- situ invertus

yes

Examination

Neck: Short, stiff

Document omovertebral bar, shoulder movement, abduction power (medial wing + superomedial angle move superiorly → glenoid pointed inferiorly)

Shoulder abduction: movement only coming from GHJ, no STJ movement

Look for UL abnormalities (VACTERL), periscapular muscle fibrosis / weakened, check distal NV status (syringomyelia)

Look for scoliosis/ kyphosis/ back pitting (spina bifida)

yes

Xray

Omovertebral bar (bar best seen on lateral/ oblique Xray of C spine)

Clavicle uptilt

High riding scapula

Small scapula (Transverse > vertical)

Inferomedial corner medial tilt + glenoid downward tilt

Association with Klippel Feil: C spine failure of segmentation, C1/2 instability, Cranial settling

Need CT and MRI spine to look for bar and spinal problem such as dural ectasia or diastematomyelia

Also cardiac and renal workup, also rule out other C spine problems

yes

Staging:

Caverndish

Very mild, not detectable in clothing

Mild 1-2cm higher

Moderate 2-5cm higher

Severe >5cm higher (superior angle near occiput)

yes

Management

OT if 1. Cosmetic problem, 2. Abduction < 120 @ 3-8yo (too late more nerve injury)

yes

Aims:

1. Excise omovertebral bar + superior trapezius release at C4

2. Relocation of scapula

Woodward (detachment and distal advancement of the midline origin of parascapular muscles)

Green (extraperiosteal detachment of periscapular muscle, then hold the scapula in its new position with spring wire traction and spica casting for 3 weeks)

Both improve 50deg

3. Clavicle morselized osteotomy → preserve periosteum (decrease brachial plexus injury)

→ Principle is to improve shoulder abduction (will improve 30 deg, Caverndish by 1 grade)

——

Congenital undescended scapula

From 4-6th C spine (pharyngeal arch), caudal migration to T2-7 during 9th to 12th weeks of gestation

yes

Interruption of embryonic subclavian blood supply

10-30% bilateral

Present in 1/3 Klippel-Feil

Association: scoliosis, spinal dysraphism, VACTERL

Klippel Feil Syndrome (SGM 1 gene at chromosome 8)

Congenital condition with fusion of more than two cervical vertebra

yes

Classical Triad

Low posterior hair line

Short webbed neck

Decreased cervical movement (normal F/E, decrease side bending)

(Hearing loss, micrognathia, torticollis, scoliosis)

yes

Xray triad

Unsegmented cervical spine (commonest C2/3)

ADI, C1/2 instability

Adjacent segment degeneration

(may also have basilar invagination)

If involve C2 → no contact sports

If lower → usually asymptomatic, until adjacent levels degenerate (100%)→ contact sports ok

OT if 1. pain, 2. instability, 3. neurology

Check/perform check CVS system and urogenital system before OT

070. Sprengel shoulder

Clean FRCS notes with inline figures

Clinical photo showing patient with asymmetrical scapula

Associations:

Examination

Xray

Staging:

Management

Aims:

Interruption of embryonic subclavian blood supply

Classical Triad

Xray triad

Direct Q&A

Neck: Short, stiff

Raw speaker-note hierarchy audit

Improved semantic hierarchy used for the study notes