Multiple
Symmetrical
Fractures
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JOHN
of Bone
P. FULKERSON’
AND
of Unresolved
M. B. OZONOFF2
were within
The clinical, laboratory, and radiologic picture of a unique patient with symmetrical fractures of the anterior tibial midshafts, dorsal ulnar cortices, and lateral metatarsals is presented. The findings are not compatible with a diagnosis of osteogenesis imperfecta or osteomalacia despite osseous fragility. The elevated serum pyrophosphate and low urine phosphate content suggest that abnormalities in phosphate metabolism may have contributed to the formation of bone which is biochemically and structurally deficient.
Etiology
normal
limits.
The serum
alkaline
phosphatase
was
208 and 146 lU (normal, 20-265 in his age group). The pyrophosphate level was 129 j.tg/100 ml (normal, 40-80).
pyrophosphate tions
gave
was within
calcium
values
and phosphates urine
and
aspirate
(normal,
900-1
decalcified
was
84
were
and
mg/24
within
hr (normal, normal
0-10)
300). The
25-77).
limits.
A bone
Serum marrow
unremarkable.
A transiliac Osseous fragility occurs in diverse childhood disorders of bone including osteogenesis impenfecta [1], hypophosphatasia [2], osteoporosis [3] homocystinu na [4], Cushing’s syndrome [5], rickets and osteomalacia [6], and disuse atrophy. The characteristics of these disordens generally are well described. However, the occurmence of multiple symmetrical fractures has not been reported except in osteomalacia [6]. This report describes a child with symmetrical fractunes of the ulnas, tibias, and metatansals sustained from minimal or unnoticed trauma at various times over a 3 year period. Evaluation of this patient at Newington Children’s Hospital failed to demonstrate the clinical and madiologic picture of osteogenesis imperfecta, osteoma-
Two 24 hr urine collec-
of 475 mg and 326 mg (normal,
carotene
was
limits.
1 20 mg
hydroxyproline
cortisol
normal of 75 mg
serum Urine
bone section
biopsy
was
performed
undecalcified
,
and examined
section
and surface measurements. The general with normal appearance of the trabeculae,
by
microradiograph,
,
pattern Iacunae,
was normal and surface
,
lacia,
or any
other
known
bone
Case
The patient, a 10-year-old descent, was born after a 2,265
g.
tion active.
of
the
ulnas
gave
rise
to
normally.
slight
swelling;
however, radiographs showed fractures through only one cortex of each ulna, and immobilization was not needed. There was no association between crutch use and these fractures. The right tibial fracture resulted from a twisted ankle and caused limping for several days but never required a cast. The left tibial fracture occurred when a baseball bat was thrown inadvertently and landed on the patient’s left leg; it required plaster immobilization.
Fractures
suIted from normal after short periods Physical
of
the
fourth
vigorous activity of immobilization.
examination
revealed
and
fifth
metatarsals
and became
a child
re-
asymptomatic
of normal
stature
with
normal skin and sclerae. The teeth were neither translucent nor decayed. The joints were supple. No stigmata of osteogenesis imperfecta were noted. The serum calcium, phosphate, total protein, and albumin Received January 17, 1977; accepted after revision I Department of Orthopedics, Newington Children’s School of Medicine, New Haven, Connecticut 06510. 2 Department of Radiology. Newington Children’s Am J Ro.ntgenol
129:313-316,
August 1977
May 3, 1977. Hospital, Newington, Hospital,
Newington,
biopsy
age group;
there
was
normal 16%
thought
mineralization.
total
osteoid
to
was no evidence
osteogenesis
m) sections. The amount of forma-
be
of
normal
The which for
this
of osteomalacia,
imperfecta,
or abnormal
Radiologic
Findings
surface 70%
was
patient’s
osteopo-
mineral
distribu-
Tibias (figs. lA-iD). The anterior cortex of each tibia was thickened, with sclerosis extending from the lower borden of the anterior tibial eminence into the lower shaft. In the midshaft of the tibia at the point of maximal sclerotic reaction theme was a cleft within the cortex extending through slightly more than half the diameter of the shaft; the bone production had increased the diameten at this point. Within the lucent cleft, small sclerotic spicules were present. The levels of the incomplete fractunes were identical on each side. No change was noted in these fractures oven a 2 year period. Ulnas (figs. 1E and iF). Theme were sclerotic cortical thickenings in the proximal posterior cortices of the ulnas, similar in appearance to those in the tibias. A lucent cleft was noted within the thickest portion of the sclenosis in each of these incomplete fractures, and the fractures were almost exactly the same distance from the olecranon on each side. The fracture was more prominent on the might than on the left, occupying approximately half of the shaft diameter. Metatarsals (fig. 2). incomplete fractures were seen in the lateral cortices of the right fourth and fifth proximal metatamsals and the left fifth proximal metatarsal. Two of these fractures healed permanently and one later ref mactuned in the same place. Skeletal Survey (fig. 3). The skull was normal without any sutumal bones or other abnormalities. Decreased contour modeling and internal metaphyseal striation of
no family history of fracture or bony abnormality. year period preceding admission he had sustained fractures of both proximal ulnas, midshaft of each lateral metatarsals of both feet. In each case, the trauma preceding the fracture was inapparent or
Fractures
and
revealed
and thin (5-6 a moderate
tion.
male of Puerto Rican and Polish month gestation and weighed proceeded
thick (85 Mm) demonstrated
resorption
The
rosis,
disorder.
milestones
and
measurements
Report
His developmental
There was Within a 3 symmetrical tibia, and amount of minimal.
7’/2
lining cells in both microradiographs
Connecticut Connecticut
313
06111
,
and Section
061 1 1 . Address
reprint
of Orthopedic requests
Surgery,
Yale University
to M. B. Ozonoff.
FULKERSON
Downloaded from www.ajronline.org by 117.253.227.123 on 10/07/15 from IP address 117.253.227.123. Copyright ARRS. For personal use only; all rights reserved
314
Fig. 1 -A and B, Right and left tibias, age 9. Note symmetrical lucencies views of right and left tibias showing incomplete fractures involving only incomplete fractures of posterior cortices with adjacent hyperostosis.
the distal femurs were noted. No other abnormalities were detected elsewhere in the skeletal survey. The metacarpal cortical thickness was within 1 SD of normal for
with
the
patient’s
age
chronological
[7],
and
bone
age
was
consistent
age. Discussion
The basic problem in this patient was skeletal fragility resulting in fractures from relatively insignificant stresses. If osteogenesis impenfecta is defined broadly as a condition in which the bones are imperfect, our patient certainly fits. However, he definitely did not have classic osteogenesis imperfecta with the osseous fragility, blue sclerae, deafness, and heritable characteristics that have been described [8]. In the 22 cases of mild osteogenesis imperfecta
described
by
Bauze
et
al.
[9],
all
had
blue
sclemae; our patient did not. Similarly, other possibilities may be eliminated. The characteristic madiologic findings of juvenile osteopomosis with vertebral compression and loss of trabeculation [3] were not present. The symmetrical nature of the fractures was similar to that described by Milkman [6]. A later analysis of Milkman syndrome by Faimbank [2] suggests that this condi-
AND
OZONOFF
crossing midshafts with sclerosis above and below fissures. C and 0, Lateral anterior cortices. E and F, Right and left ulnas, age 10, showing symmetrical
tion is associated with osteomalacia. In our patient’s age group, osteomalacia would appear as rickets, which he did not have. The bone did not demonstrate widened osteoid seams on other stigmata of osteomalacia, and nadiognaphs were not consistent with a diagnosis of Osteomalacia on rickets. North [10] described an otherwise healthy adult with multiple asymmetrical spontaneous fractures who did not show histologic evidence of osteomalacia. North’s case is similar to ours except for the fracture distribution. Stress fractures in children are well described. They are sometimes bilateral, as were 12% of the tibial stress fractures reported by Wilson and Katz[1 1] Stress fmactunes often occur in the tibial midshaft, calcaneus, femum, and metatarsals [11-13]. Stress fractures occur at sites of continued stress; the fracture locations in this patient would fit this requirement except for those in the ulnas. Although this patient’s fractures are similar to stress fractures, the frequent occurrence, multiple sites, and symmetry suggest an intrinsic defect in bone structune. The elevated serum pynophosphate in our patient wanrants some consideration in view of the apparent inhibition of calcification by pyrophosphate [14]. Solomons .
Downloaded from www.ajronline.org by 117.253.227.123 on 10/07/15 from IP address 117.253.227.123. Copyright ARRS. For personal use only; all rights reserved
MULTIPLE
SYMMETRICAL
FRACTURES
OF
315
BONE
Fig. 2.-A, Left foot, age 10. Note healing fracture of fifth metatarsal. (Lateral projection showed this to involve only posterior cortex.) B, Right foot, age 8, showing incomplete fracture of the fifth metatarsal. C, Right foot, age 10. Fifth metatarsal fracture has healed with only faint residual trabecular thickening . Note healing incomplete fracture of fourth metatarsal.
. ----
cortical
koletalsurvey.A, modeling.
.snormal
.
osutura
bones.B,
L
and Stynem [15] demonstrated elevation of serum pyrophosphate in 19 patients with osteogenesis imperfecta, but it is likely that pyrophosphate levels are elevated in other metabolic disorders as well. Pyrophosphate may
.
sshowlongitut
rabecular
st iations
associal
be important in the modulation of calcification, and the elevated pyrophosphate level in our patient suggests the possibility of a defect at this level of metabolism. The decreased urinary excretion of phosphate sug-
316
FULKERSON
gests an abnormality in phosphate regulation. The slightly elevated urine hydmoxyproline may be related to fracture healing and cannot be considered relevant to the underlying bone disorder. Urinary hydroxyproline is a
Downloaded from www.ajronline.org by 117.253.227.123 on 10/07/15 from IP address 117.253.227.123. Copyright ARRS. For personal use only; all rights reserved
nonspecific
indicator
of collagen
breakdown
or turnover
[16]. REFERENCES 1 Albnight JA, Grunt JA: Studies of patients with osteogenesis imperfecta. J Bone Joint Surg [Am] 53:1415-1425, 1971 .
2.
Fainbank HAT: An Atlas of General Affections ton. Edinburgh, Livingstone, 1951
of the Skele-
,
Joint
Surg
[Br]
38:418-433,
OZONOFF the differential
1956
6. Milkman LA: Pseudofractures (hunger osteopathy, late rickets, osteomalacia). Report of a case. Am J Roentgenol 24:29-37, 1930 7. Garn SM, Poznanski AK, Nagy JM: Bone measurement in
diagnosis
of osteopenia
Radiology 100 : 509-518, 1971 8. King JD, Bobechko WP: Osteogenesis
paedic
description and surgical [BrJ 53:72-89, 1971 9. Bauze
RJ, Smith
A, Francis
review.
MJO:
and osteoporosis. imperfecta: an orthoJ Bone Joint Surg
A new look at osteogene-
sis imperfecta. J Bone Joint Surg (Br] 57:2-12, 1975 10. North KAK: Multiple stress fractures simulating osteomalacia. Am J Roentgenol 97 : 672-675, 1966 11 Wilson ES Jr, Katz FN: Stress fractures: An analysis of 250 consecutive cases. Radiology 92 : 481-486, 1969 12. Devas MB: Stress fractures in children. J Bone Joint Surg .
[Br]
3. Jackson WPU: Osteoporosis of unknown cause in younger people. J Bone Joint Surg [Br] 40 : 420-441 1958 4. McKusick VA: Heritable Disorders of Connective Tissue, 4th ed. St. Louis, Mosby, 1972 5. Sissons HA: The osteoporosis of Cushing’s syndrome. J Bone
AND
45:529-541
,
1963
13. Savoca CJ: Stress fractures: radiographic signs. Radiology 14.
a classification 100:519-524,
Fleisch H: Role of nucleation and inhibition C!in Orthop 32 : 1 70-1 80, 1964
of the earliest 1971 of calcification.
15. Solomons CC, Styner J: Osteogenesis imperfecta: Effect of magnesium admi nistration on pyrophosphate metabolism. Calcif Tissue Res 3 :31 8-326, 1969 16. Prockop DJ, Kivirikko, KI: Relationship of hydroxyproline excretion in urine to collagen metabolism. Ann Intern Med 66 : 1243-1266, 1967
Symmetrical
Fractures
Downloaded from www.ajronline.org by 117.253.227.123 on 10/07/15 from IP address 117.253.227.123. Copyright ARRS. For personal use only; all rights reserved
JOHN
of Bone
P. FULKERSON’
AND
of Unresolved
M. B. OZONOFF2
were within
The clinical, laboratory, and radiologic picture of a unique patient with symmetrical fractures of the anterior tibial midshafts, dorsal ulnar cortices, and lateral metatarsals is presented. The findings are not compatible with a diagnosis of osteogenesis imperfecta or osteomalacia despite osseous fragility. The elevated serum pyrophosphate and low urine phosphate content suggest that abnormalities in phosphate metabolism may have contributed to the formation of bone which is biochemically and structurally deficient.
Etiology
normal
limits.
The serum
alkaline
phosphatase
was
208 and 146 lU (normal, 20-265 in his age group). The pyrophosphate level was 129 j.tg/100 ml (normal, 40-80).
pyrophosphate tions
gave
was within
calcium
values
and phosphates urine
and
aspirate
(normal,
900-1
decalcified
was
84
were
and
mg/24
within
hr (normal, normal
0-10)
300). The
25-77).
limits.
A bone
Serum marrow
unremarkable.
A transiliac Osseous fragility occurs in diverse childhood disorders of bone including osteogenesis impenfecta [1], hypophosphatasia [2], osteoporosis [3] homocystinu na [4], Cushing’s syndrome [5], rickets and osteomalacia [6], and disuse atrophy. The characteristics of these disordens generally are well described. However, the occurmence of multiple symmetrical fractures has not been reported except in osteomalacia [6]. This report describes a child with symmetrical fractunes of the ulnas, tibias, and metatansals sustained from minimal or unnoticed trauma at various times over a 3 year period. Evaluation of this patient at Newington Children’s Hospital failed to demonstrate the clinical and madiologic picture of osteogenesis imperfecta, osteoma-
Two 24 hr urine collec-
of 475 mg and 326 mg (normal,
carotene
was
limits.
1 20 mg
hydroxyproline
cortisol
normal of 75 mg
serum Urine
bone section
biopsy
was
performed
undecalcified
,
and examined
section
and surface measurements. The general with normal appearance of the trabeculae,
by
microradiograph,
,
pattern Iacunae,
was normal and surface
,
lacia,
or any
other
known
bone
Case
The patient, a 10-year-old descent, was born after a 2,265
g.
tion active.
of
the
ulnas
gave
rise
to
normally.
slight
swelling;
however, radiographs showed fractures through only one cortex of each ulna, and immobilization was not needed. There was no association between crutch use and these fractures. The right tibial fracture resulted from a twisted ankle and caused limping for several days but never required a cast. The left tibial fracture occurred when a baseball bat was thrown inadvertently and landed on the patient’s left leg; it required plaster immobilization.
Fractures
suIted from normal after short periods Physical
of
the
fourth
vigorous activity of immobilization.
examination
revealed
and
fifth
metatarsals
and became
a child
re-
asymptomatic
of normal
stature
with
normal skin and sclerae. The teeth were neither translucent nor decayed. The joints were supple. No stigmata of osteogenesis imperfecta were noted. The serum calcium, phosphate, total protein, and albumin Received January 17, 1977; accepted after revision I Department of Orthopedics, Newington Children’s School of Medicine, New Haven, Connecticut 06510. 2 Department of Radiology. Newington Children’s Am J Ro.ntgenol
129:313-316,
August 1977
May 3, 1977. Hospital, Newington, Hospital,
Newington,
biopsy
age group;
there
was
normal 16%
thought
mineralization.
total
osteoid
to
was no evidence
osteogenesis
m) sections. The amount of forma-
be
of
normal
The which for
this
of osteomalacia,
imperfecta,
or abnormal
Radiologic
Findings
surface 70%
was
patient’s
osteopo-
mineral
distribu-
Tibias (figs. lA-iD). The anterior cortex of each tibia was thickened, with sclerosis extending from the lower borden of the anterior tibial eminence into the lower shaft. In the midshaft of the tibia at the point of maximal sclerotic reaction theme was a cleft within the cortex extending through slightly more than half the diameter of the shaft; the bone production had increased the diameten at this point. Within the lucent cleft, small sclerotic spicules were present. The levels of the incomplete fractunes were identical on each side. No change was noted in these fractures oven a 2 year period. Ulnas (figs. 1E and iF). Theme were sclerotic cortical thickenings in the proximal posterior cortices of the ulnas, similar in appearance to those in the tibias. A lucent cleft was noted within the thickest portion of the sclenosis in each of these incomplete fractures, and the fractures were almost exactly the same distance from the olecranon on each side. The fracture was more prominent on the might than on the left, occupying approximately half of the shaft diameter. Metatarsals (fig. 2). incomplete fractures were seen in the lateral cortices of the right fourth and fifth proximal metatamsals and the left fifth proximal metatarsal. Two of these fractures healed permanently and one later ref mactuned in the same place. Skeletal Survey (fig. 3). The skull was normal without any sutumal bones or other abnormalities. Decreased contour modeling and internal metaphyseal striation of
no family history of fracture or bony abnormality. year period preceding admission he had sustained fractures of both proximal ulnas, midshaft of each lateral metatarsals of both feet. In each case, the trauma preceding the fracture was inapparent or
Fractures
and
revealed
and thin (5-6 a moderate
tion.
male of Puerto Rican and Polish month gestation and weighed proceeded
thick (85 Mm) demonstrated
resorption
The
rosis,
disorder.
milestones
and
measurements
Report
His developmental
There was Within a 3 symmetrical tibia, and amount of minimal.
7’/2
lining cells in both microradiographs
Connecticut Connecticut
313
06111
,
and Section
061 1 1 . Address
reprint
of Orthopedic requests
Surgery,
Yale University
to M. B. Ozonoff.
FULKERSON
Downloaded from www.ajronline.org by 117.253.227.123 on 10/07/15 from IP address 117.253.227.123. Copyright ARRS. For personal use only; all rights reserved
314
Fig. 1 -A and B, Right and left tibias, age 9. Note symmetrical lucencies views of right and left tibias showing incomplete fractures involving only incomplete fractures of posterior cortices with adjacent hyperostosis.
the distal femurs were noted. No other abnormalities were detected elsewhere in the skeletal survey. The metacarpal cortical thickness was within 1 SD of normal for
with
the
patient’s
age
chronological
[7],
and
bone
age
was
consistent
age. Discussion
The basic problem in this patient was skeletal fragility resulting in fractures from relatively insignificant stresses. If osteogenesis impenfecta is defined broadly as a condition in which the bones are imperfect, our patient certainly fits. However, he definitely did not have classic osteogenesis imperfecta with the osseous fragility, blue sclerae, deafness, and heritable characteristics that have been described [8]. In the 22 cases of mild osteogenesis imperfecta
described
by
Bauze
et
al.
[9],
all
had
blue
sclemae; our patient did not. Similarly, other possibilities may be eliminated. The characteristic madiologic findings of juvenile osteopomosis with vertebral compression and loss of trabeculation [3] were not present. The symmetrical nature of the fractures was similar to that described by Milkman [6]. A later analysis of Milkman syndrome by Faimbank [2] suggests that this condi-
AND
OZONOFF
crossing midshafts with sclerosis above and below fissures. C and 0, Lateral anterior cortices. E and F, Right and left ulnas, age 10, showing symmetrical
tion is associated with osteomalacia. In our patient’s age group, osteomalacia would appear as rickets, which he did not have. The bone did not demonstrate widened osteoid seams on other stigmata of osteomalacia, and nadiognaphs were not consistent with a diagnosis of Osteomalacia on rickets. North [10] described an otherwise healthy adult with multiple asymmetrical spontaneous fractures who did not show histologic evidence of osteomalacia. North’s case is similar to ours except for the fracture distribution. Stress fractures in children are well described. They are sometimes bilateral, as were 12% of the tibial stress fractures reported by Wilson and Katz[1 1] Stress fmactunes often occur in the tibial midshaft, calcaneus, femum, and metatarsals [11-13]. Stress fractures occur at sites of continued stress; the fracture locations in this patient would fit this requirement except for those in the ulnas. Although this patient’s fractures are similar to stress fractures, the frequent occurrence, multiple sites, and symmetry suggest an intrinsic defect in bone structune. The elevated serum pynophosphate in our patient wanrants some consideration in view of the apparent inhibition of calcification by pyrophosphate [14]. Solomons .
Downloaded from www.ajronline.org by 117.253.227.123 on 10/07/15 from IP address 117.253.227.123. Copyright ARRS. For personal use only; all rights reserved
MULTIPLE
SYMMETRICAL
FRACTURES
OF
315
BONE
Fig. 2.-A, Left foot, age 10. Note healing fracture of fifth metatarsal. (Lateral projection showed this to involve only posterior cortex.) B, Right foot, age 8, showing incomplete fracture of the fifth metatarsal. C, Right foot, age 10. Fifth metatarsal fracture has healed with only faint residual trabecular thickening . Note healing incomplete fracture of fourth metatarsal.
. ----
cortical
koletalsurvey.A, modeling.
.snormal
.
osutura
bones.B,
L
and Stynem [15] demonstrated elevation of serum pyrophosphate in 19 patients with osteogenesis imperfecta, but it is likely that pyrophosphate levels are elevated in other metabolic disorders as well. Pyrophosphate may
.
sshowlongitut
rabecular
st iations
associal
be important in the modulation of calcification, and the elevated pyrophosphate level in our patient suggests the possibility of a defect at this level of metabolism. The decreased urinary excretion of phosphate sug-
316
FULKERSON
gests an abnormality in phosphate regulation. The slightly elevated urine hydmoxyproline may be related to fracture healing and cannot be considered relevant to the underlying bone disorder. Urinary hydroxyproline is a
Downloaded from www.ajronline.org by 117.253.227.123 on 10/07/15 from IP address 117.253.227.123. Copyright ARRS. For personal use only; all rights reserved
nonspecific
indicator
of collagen
breakdown
or turnover
[16]. REFERENCES 1 Albnight JA, Grunt JA: Studies of patients with osteogenesis imperfecta. J Bone Joint Surg [Am] 53:1415-1425, 1971 .
2.
Fainbank HAT: An Atlas of General Affections ton. Edinburgh, Livingstone, 1951
of the Skele-
,
Joint
Surg
[Br]
38:418-433,
OZONOFF the differential
1956
6. Milkman LA: Pseudofractures (hunger osteopathy, late rickets, osteomalacia). Report of a case. Am J Roentgenol 24:29-37, 1930 7. Garn SM, Poznanski AK, Nagy JM: Bone measurement in
diagnosis
of osteopenia
Radiology 100 : 509-518, 1971 8. King JD, Bobechko WP: Osteogenesis
paedic
description and surgical [BrJ 53:72-89, 1971 9. Bauze
RJ, Smith
A, Francis
review.
MJO:
and osteoporosis. imperfecta: an orthoJ Bone Joint Surg
A new look at osteogene-
sis imperfecta. J Bone Joint Surg (Br] 57:2-12, 1975 10. North KAK: Multiple stress fractures simulating osteomalacia. Am J Roentgenol 97 : 672-675, 1966 11 Wilson ES Jr, Katz FN: Stress fractures: An analysis of 250 consecutive cases. Radiology 92 : 481-486, 1969 12. Devas MB: Stress fractures in children. J Bone Joint Surg .
[Br]
3. Jackson WPU: Osteoporosis of unknown cause in younger people. J Bone Joint Surg [Br] 40 : 420-441 1958 4. McKusick VA: Heritable Disorders of Connective Tissue, 4th ed. St. Louis, Mosby, 1972 5. Sissons HA: The osteoporosis of Cushing’s syndrome. J Bone
AND
45:529-541
,
1963
13. Savoca CJ: Stress fractures: radiographic signs. Radiology 14.
a classification 100:519-524,
Fleisch H: Role of nucleation and inhibition C!in Orthop 32 : 1 70-1 80, 1964
of the earliest 1971 of calcification.
15. Solomons CC, Styner J: Osteogenesis imperfecta: Effect of magnesium admi nistration on pyrophosphate metabolism. Calcif Tissue Res 3 :31 8-326, 1969 16. Prockop DJ, Kivirikko, KI: Relationship of hydroxyproline excretion in urine to collagen metabolism. Ann Intern Med 66 : 1243-1266, 1967
