Femoral and Lumbar Fractures During Rehabilitation for a Traumatic Spinal Cord Injury in Osteogenesis Imperfecta: A Case Presentation

Accepted Manuscript Femoral and Lumbar Fractures During Rehabilitation for a Traumatic Spinal Cord Injury in Osteogenesis Imperfecta: A Case Report Brendon S. Ross, David Ripley, Anna M. Ho, Leslie Rydberg PII:

S1934-1482(16)31092-9

DOI:

10.1016/j.pmrj.2017.04.010

Reference:

PMRJ 1894

To appear in:

PM&R

Received Date: 14 November 2016 Accepted Date: 12 April 2017

Please cite this article as: Ross BS, Ripley D, Ho AM, Rydberg L, Femoral and Lumbar Fractures During Rehabilitation for a Traumatic Spinal Cord Injury in Osteogenesis Imperfecta: A Case Report, PM&R (2017), doi: 10.1016/j.pmrj.2017.04.010. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

ACCEPTED MANUSCRIPT

Femoral and Lumbar Fractures During Rehabilitation for a Traumatic Spinal Cord Injury in Osteogenesis Imperfecta: A Case Report Brendon S. Ross1,2, David Ripley1,2, Anna M. Ho2, Leslie Rydberg1,2

RI PT

1

SC

Northwestern University/McGaw Medical Center, Department of Physical Medicine and Rehabilitation, Northwestern University Feinberg School of Medicine, Chicago, IL 2 Rehabilitation Institute of Chicago, Chicago, IL Correspondence: Brendon S Ross, Rehabilitation Institute of Chicago, 345 E Superior Street, Suite 1600, Chicago, IL 60611, [email protected] Phone:312-238-1000 Fax: 312-238-8405

Brendon S Ross DO, MS Resident Physician PGY-3 Physical Medicine and Rehabilitation

M AN U

Author Information:

TE D

David Ripley, MD, MS, CRC, FAAPM&R Medical Director, Brain Injury Medicine and Rehabilitation Medical Director, Workers’ Compensation Physician Practice Program Director, O’Bolye Fellowship in Brain Injury Medicine Attending Physician, Rehabilitation Institute of Chicago Associate Professor of Physical Medicine and Rehabilitation Northwestern University Feinberg School of Medicine

EP

Anna Marie Ho, PT, DPT2 General Rehabilitation/ Medically Complex Unit Rehabilitation Institute of Chicago

AC C

Leslie Rydberg, MD Attending Physician, Rehabilitation Institute of Chicago Assistant Professor of Physical Medicine and Rehabilitation Northwestern University Feinberg School of Medicine

ACCEPTED MANUSCRIPT

Abstract

3

Osteogenesis Imperfecta (OI) is one of the most common inherited bone disorders.

4

These individuals are high-risk for developing fractures during their lifetime secondary

5

to bone fragility. This case presents a female with Type I OI involved in a high speed

6

motor vehicle accident resulting in a traumatic spinal cord injury (SCI) and paraplegia.

7

Inpatient rehabilitation was complicated by fractures of the femur and lumbar spine

8

which impacted her level of independence upon discharge to prevent additional

9

fractures and maintain safety. OI coupled with SCI creates a difficult combination for

10

the rehabilitation team. This case highlights the complexity of this challenge to bring

11

awareness to the rehabilitation team in order to safely maximize independence and

12

minimize and prevent unnecessary injury when designing an interdisciplinary treatment

13

plan.

14

Introduction

15

Osteogenesis imperfecta (OI) is one of the most common inherited bone disorders

16

leading to bone fragility and increased fracture risk secondary to a genetic defect in

17

collagen I formation. Fracture severity and frequency can range anywhere from a few

18

per lifetime to lethal in utero depending on OI type. Long bones of the arms and legs

19

and small bones of the hands and feet are most commonly affected. Individuals with

20

severe OI can have as many as 200 fractures within a lifetime (1,2). There is a wide

21

range of disability in people with OI and a poorer overall quality of life; however,

22

evidence in the literature has shown adults with OI are well educated [often greater than

AC C

EP

TE D

M AN U

SC

RI PT

2

2

ACCEPTED MANUSCRIPT

the general public], most are actively employed, and a majority perform their activities

24

of daily living (ADL) independently (3-5).

25

One of the leading causes of traumatic spinal cord injury (SCI) in this country is motor

26

vehicle accidents (MVA)(6). There is a paucity of literature and scattered case reports

27

over the last few decades commenting on SCI injury in OI, but there is no current

28

literature describing the functional outcomes and rehabilitation challenges in this rare

29

clinical presentation (7-9). This case report presents a 51-year-old female with Type I

30

OI involved in a high speed motor vehicle accident leading to a lumbar burst fracture

31

resulting in paraplegia. Her inpatient rehabilitation course was complicated by fractures

32

of the femur and lumbar spine during participation in an acute inpatient SCI program.

33

The following description of this unique inpatient rehabilitation course highlights the

34

importance and complexity of creating an appropriate interdisciplinary treatment plan

35

for these high-fracture risk individuals in order to safely maximize independence while

36

minimizing and preventing unnecessary morbidity.

37

Case Presentation

38

A 51-year-old female with a history of Type I OI, upper extremity fractures and spinal

39

fixation with bilateral Harrington rods was a restrained passenger in a high-speed MVA

40

and suffered a traumatic burst fracture at the second lumbar vertebral level (L2) (Fig 1).

41

Although no other fractures were sustained, her trauma resulted in a concomitant T-7

42

American Spinal Injury Association (ASIA) Grade A SCI with flaccid bilateral lower

43

extremity paralysis. Given her compromised bone integrity secondary to her premorbid

AC C

EP

TE D

M AN U

SC

RI PT

23

3

ACCEPTED MANUSCRIPT

OI, she was managed non-operatively with three months bed rest for bony healing at a

45

long-term acute care facility. Prior to the accident, she did have functional limitations

46

from her OI and fracture history necessitating the use of a power wheelchair for

47

community ambulation. She was admitted to inpatient rehabilitation in a Model System

48

spinal cord injury rehabilitation program.

49

Long term goals for self-care skills on admission were targeted for independence with

50

grooming and upper body dressing, and performing bathing, lower body dressing, as

51

well as toileting with a slide board and drop arm commode with minimal assistance

52

from a family member or caretaker. In regards to mobility, slide board transferring to

53

her wheelchair and other surfaces with minimal assistance was an initial goal, with

54

power wheelchair use for community ambulation.

SC

M AN U

Femoral Fracture

TE D

55

RI PT

44

Approximately one week after admission, she was performing supported ring sit with

57

leg loops to practice lower extremity dressing when she heard an audible “pop” in her

58

right leg. Plain film X-ray of the right hip confirmed an angulated subtrochanteric

59

femur fracture (Fig 2). She was evaluated by the orthopedic surgery service who

60

decided to manage the fracture non-operatively with no activity restrictions. She was

61

transferred back to acute inpatient rehabilitation within twenty-four hours.

62

63

AC C

EP

56

Lumbar Fracture Her functional mobility goals were highly impacted secondary to the femur fracture

4

ACCEPTED MANUSCRIPT

prompting precautions including 1) no supported ring sit, 2) no slide board transfers to

65

prevent upper extremity fracture/injury, 3) mechanical lift for all bed/chair transfers,

66

and 4) careful monitoring and positioning of lower extremities at all times. She

67

remained at a minimum assistance level rolling in bed for bladder, bowel and linen

68

management. While performing bed rolling for bladder management and attempting to

69

position herself lateral recumbent, she had an onset of severe lower back pain.

70

Subsequent imaging confirmed an acute compression deformity in the L3 vertebral

71

body. The spine surgery service was consulted and decided to manage the fracture non-

72

operatively with no activity restrictions. She required a brief hospitalization for pain

73

control prior to being transferred back to acute inpatient rehabilitation. Strict log rolling

74

method was enacted upon return to rehabilitation for additional spinal precautions and

75

safety.

SC

M AN U

TE D

76

RI PT

64

Functional Implications

After the femur fracture, precautions were implemented secondary to her high-fracture

78

risk (Table 1). To achieve higher functional independence, she would have to accept a

79

higher risk of fracture. To minimize fracture risk, she would have to do safer transfers

80

(mechanical lift) and avoid postures causing stress which would limit her independence.

81

Several of her previous short and long term functional admission goals were

82

discontinued or significantly modified focusing on family training for safe transfers and

83

ADL assistance (Table 1). Additional precautions, notably strict log rolling, were

84

enacted after her lumbar fracture which was also additive to her functional limitations

85

upon discharge.

AC C

EP

77

5

ACCEPTED MANUSCRIPT

These precautions and functional limitations significantly affected how much additional

87

assistance she would require if she were to return home. Despite these, she had a very

88

good support system at home and her family was able to provide the additional

89

assistance required for self-care, bladder/bowel management and transfers with the

90

mechanical lift. Although she made limited functional gains despite her six-week time

91

course in acute inpatient rehabilitation, appropriate risk-factor modifications were

92

employed, family training was successful and safety awareness to maintain a fracture-

93

free lifestyle was accomplished.

94

Discussion

95

Unlike other therapy approaches in patients attempting to return to normal movement

96

patterns, many SCI patients will have to learn novel ways and techniques from

97

therapists to perform everyday movements and tasks. This most often requires the long

98

process of learning to use and strengthen your upper extremities to assist with new

99

weight-bearing requirements, as well as several functional postures that are key to

TE D

M AN U

SC

RI PT

86

mobility and self-care. These postures may include rolling, supported short sit,

101

supported long sit, supported ring sit, prone on elbows and other functional postures.

102

There is a variety of literature supporting the efficacy and benefits of rehabilitation

103

programs in patients with OI, but the overwhelming majority of these studies are in

104

pediatric populations and these studies are without comorbid SCI (10, 11). The

105

Osteogenesis Imperfecta Foundation has published a useful guide for physical and

106

occupational therapists with many precautionary considerations discussed when

AC C

EP

100

6

ACCEPTED MANUSCRIPT

working with OI individuals, but also with a pediatric focus. Some of these include 1)

108

awareness of the person’s arms and legs at all times to avoid awkward positioning or

109

getting caught on clothing, 2) never pushing, pulling or twisting a limb with avoidance

110

of passive rotation of the head, trunk, arms and legs, 3) avoid positions of great leverage

111

that stress bones, such as hip flexion (“jack-knife” position), diagonal trunk rotation and

112

bridging exercises. Extrapolating many of these pediatric based precautions to OI with

113

SCI may be necessary at baseline for appropriate risk management.

114

guides are useful, it is also important to realize that SCI creates a plethora of challenges

115

to the typical rehabilitation program for individuals with OI and vice versa. In 1984,

116

Ohry commented on a unique case of a 30-year-old OI patient with complete paraplegia

117

from a motor cycle accident recovering at the Stoke Mandeville Hospital in the United

118

Kingdom, “After the SCI we were faced with special problems in this patient. We had

119

to prevent him from engaging in any sporting activities, such as ‘push-ups’ and other

120

strenuous exercises, because of the danger of fractures.”(9). This case clearly illustrates

121

these “special problems.”

122

The fractures experienced during the functional postures and mobility strategies which

123

are normally taught to SCI patients obligated the team to evaluate each adaptive

124

strategy to prevent fracture and ensure patient safety. The combined forces of high-

125

fracture risk and poor bone health limited her possibilities for future independence.

126

The compromised premorbid bone integrity and associated ligamentous laxity of OI

127

coupled with SCI creates a difficult combination for the rehabilitation team.

128

This report highlights the importance of rehabilitation teams understanding these

SC

RI PT

107

AC C

EP

TE D

M AN U

Although these

7

ACCEPTED MANUSCRIPT

boundaries when managing OI with SCI and appropriate risk analysis of functional

130

goals with the rehabilitation team is necessary to prevent injury. Additional therapy

131

guidelines and precautions may be warranted from a systems standpoint to further

132

improve the spinal rehabilitative care in this unique patient population. In spite of these

133

challenges, it is possible to create an appropriate rehabilitation plan for individuals with

134

the combination of OI and SCI.

SC

RI PT

129

137 138 139 140 141

REFERENCES

1. Bishop NJ, Walsh JS. Osteogenesis imperfecta in adults. Journal of Clin Invest 2014; 124(2):476-77

2. Werker LL, Eriksen EF, Falch JA. Bone mass, bone markers and prevalence of fractures in adults with osteogenesis imperfecta. Arch Osteoporos 2011; 6:31-38

TE D

136

M AN U

135

3. Werke LL, Froslie KF, Haugen L et al. A population based study of demographical variables and ability to perform activities of daily living in adults

143

with osteogenesis imperfecta. Disability Rehab 2010; 32(7):579-587

144

4. Tosi LL, Oetgen ME, Floor MK et al. Initial report of the osteogenesis

146 147

imperfecta adult natural history initiative. Orphanet Journal of Rare Diseases

AC C

145

EP

142

2015; 10:146

5. Tsimicalis A, Denis-Larocque G, Michalovic A et al. The psychosocial

148

experience of individuals living with osteogenesis imperfecta: a mixed-methods

149

systematic review. Qual Life Research 2016; 25(8):1877-96

8

ACCEPTED MANUSCRIPT

150

6.

Jain NB, Ayers GD, Peterson EN, Harris MB, Morse L, O'Connor KC, Garshick E. Traumatic spinal cord injury in the United States, 1993-2012. JAMA. 2015

152

Jun 9; 313(22):2236-43.

153

RI PT

151

7. Leng LZ, Shajari M, Hartl R. Management of Acute Cervical Compression Fractures in Two Patients With Osteogenesis Imperfecta. Spine 2010;

155

35(22):1248-1252

157 158 159 160

8. Ziv I, Rang M, Hoffman HJ. Paraplegia in Osteogenesis Imperfecta. Journal of Bone and Joint Surgery 1983; 65(2):184-85

M AN U

156

SC

154

9. Ohry A, Frankel HL. Rehabilitation After Spinal Cord Injuries Complicated by Previous Lesions. Paraplegia 184; 22:291-296

10. Hoyer-Kuhn H, Semler O, Stark C et al. A specialized rehabilitation approach improves mobility in children with osteogenesis imperfecta. J Musculoskelet

162

Neuronal Interact 2014; 14(4):445-453.

163

TE D

161

11. Osteogenesis Imperfecta Foundation. Therapeutic Strategies for Osteogenesis Imperfecta: A guide for physical therapists and Occupations Therapists.

165

National Institutes of Health: Osteoporosis and Related Bone Diseases ~

166

National Resource Center

168 169

AC C

167

EP

164

170 171

9

ACCEPTED MANUSCRIPT

FIGURE LEGEND

173

Figure 1. Computerized Tomography lumbar spine demonstrating an acute traumatic

174

burst fracture of the second lumbar vertebral level.

RI PT

172

175

Figure 2. Plain film X-Ray of the right hip demonstrates an angulated subtrochanteric

177

femur fracture.

SC

176

178

M AN U

179 180 181

AC C

EP

TE D

182

10

ACCEPTED MANUSCRIPT

TABLE 1. Functional Status/Rehabilitation Goals Admission FIMS*

Admission Goals

Post Fracture Goal ∆

RI PT

PHYSICAL THERAPY

Discharge FIMS*

Moderate Assistance

Close Supervision

Moderate Assistance

Maximal Assistance

Bed/Wheelchair Transfers

Moderate Assistance

Minimal Assistance

Total Assistance

Total Assistance

Toilet Transfer

Not Assessed

Minimal Assistance

Wheelchair Mobility

Modified Independence

Modified Independence

SC

Bed Mobility

Does Not Occur

Modified Independence

Modified Independence

M AN U

Discontinued

OCCUPATIONAL THERAPY Moderate Assistance

Upper Body Dressing

Setup

Minimal Assistance

Minimal Assistance (bed-level)

Moderate Assistance (bed-level)

Modified Independent

Modified Independent

Minimal Assistance

TE D

Full Body Bathing

Lower Body Dressing

Minimal Assistance

Maximal Assistance

Maximal Assistance

Total Assistance

Minimal Assistance

Maximal Assistance

Maximal Assistance

EP

Toileting

Total Assistance

Moderate Assistance

Minimal Assistance

Discontinued

Does Not Occur

Tub/Shower Transfer

Total Assistance

Moderate Assistance

Discontinued

Does Not Occur

AC C

Toilet Transfer

HIGH-RISK FRACTURE PRECAUTIONS

• • • • • •

No supported ring sit No slide board transfers to prevent upper extremity fracture/injury Avoid positions of excessive twisting, bending and passive rotation of lower extremities Mechanical Lift for all bed/chair transfers Careful monitoring of positioning of lower extremities during mechanical lift transfers Strict whole body log rolling during bladder/bowel/linen management to prevent axial spine stress

*FUNCTIONAL INDEPENDENCE MEASUREMENT SCORE

ACCEPTED MANUSCRIPT

AC C

EP

TE D

M AN U

SC

RI PT

Figure 1.

ACCEPTED MANUSCRIPT

AC C

EP

TE D

M AN U

SC

RI PT

Figure 2.