Abstract Introduction Osteopetrosis introduces technical limitations to the traditional treatment of fracture management that may be minimised with specific pre-operative planning. Extreme care and caution are required when drilling, reaming, or inserting implants in patients with osteopetrosis. Caution must be exercised throughout the postoperative course when these patients are at greatest risk for device failure or further injury. Case presentation We present our experience of treating such a fracture where a patient presented with a non-united fracture of the humerus. The bone was already osteoporotic. We successfully used a new technique which has not been described in the literature before. This included the use of a high-speed drill to prepare the bone for screw fixation. Bone healing was augmented with bone morphogenic protein. Conclusion This technique can give invaluable experience to surgeons who are involved in treating these types of fracture.</p

A Chhabra

Amit Kapoor

David JC Burton

DG Armstrong

F Shapiro

H Albers-Schonberg

Imran Rafiq

J Bollerslev

John F Haines

JP Strickland

JW Milgram

MS Kocher

N Dahl

R Derner

English

PubMed

Springer - Publisher Connector

BioMed CentralJournal of Medical Case Reports
ssOpen AcceCase report
A new modality of treatment for non-united fracture of the 
humerus in a patient with osteopetrosis: a case report
Imran Rafiq*1,2, Amit Kapoor1, David JC Burton1 and John F Haines1
Address: 1Upper Limb Unit, Wrightington Hospital, Hall Lane, Appley Bridge, Wigan, Lancashire, WN6 9EP, UK and 2197, Berberis House, 
Highfield Road, Feltham, TW13 4GS, UK
Email: Imran Rafiq* - imranrafiq@doctors.net.uk; Amit Kapoor - dramitkapoor@doctors.net.uk; David JC Burton - davburton@glos.nhs.uk; 
John F Haines - JFHaines@uhls.org.uk
* Corresponding author    
Abstract
Introduction: Osteopetrosis introduces technical limitations to the traditional treatment of
fracture management that may be minimised with specific pre-operative planning. Extreme care and
caution are required when drilling, reaming, or inserting implants in patients with osteopetrosis.
Caution must be exercised throughout the postoperative course when these patients are at
greatest risk for device failure or further injury.
Case presentation: We present our experience of treating such a fracture where a patient
presented with a non-united fracture of the humerus. The bone was already osteoporotic. We
successfully used a new technique which has not been described in the literature before. This
included the use of a high-speed drill to prepare the bone for screw fixation. Bone healing was
augmented with bone morphogenic protein.
Conclusion: This technique can give invaluable experience to surgeons who are involved in
treating these types of fracture.
Introduction
Osteopetrosis is a rare skeletal condition first described by
German radiologist Heinrich Albers-Schonberg in 1904
[1]. The condition is characterised by skeletal osteosclero-
sis caused by aberrant osteoclast-mediated bone resorp-
tion. Management of patients with osteopetrosis requires
a comprehensive approach to characteristic clinical prob-
lems including metabolic abnormalities, fractures,
deformities, back pain, bone pain, osteomyelitis and neu-
rological sequelae [2]. Although fractures can be managed
conservatively, they can be challenging when considering
the internal fixation required to rectify non-union and
mal-union. There have been many documented technical
difficulties in operative management for fixation of frac-
tures in these patients. We used a high-speed drill and
bone morphogenic protein to treat a patient with a non-
united fracture of the proximal humerus. We have not
found any evidence of the use of this technique in the
medical literature to treat fractures in osteopetrotic
patients.
Case presentation
A 48-year-old male general physician was referred to our
unit from a neighbouring hospital with a non-united frac-
ture of the right proximal humerus (Figure 1). The injury
was sustained as a result of falling down stairs and was ini-
tially managed conservatively for 3 months. There was
minimal callus formation with symptoms of fracture
Published: 13 January 2009
Journal of Medical Case Reports 2009, 3:15 doi:10.1186/1752-1947-3-15
Received: 6 February 2008
Accepted: 13 January 2009
This article is available from: http://www.jmedicalcasereports.com/content/3/1/15
© 2009 Rafiq et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3
(page number not for citation purposes)
Journal of Medical Case Reports 2009, 3:15 http://www.jmedicalcasereports.com/content/3/1/15union and there was a potential stress line in the distal
fragment 4 cm below the fracture. His past history com-
prised osteopetrosis resulting in fractures of the left femur,
left radius and ulna, the latter managed operatively. Open
reduction and internal fixation was decided for the frac-
ture of the humerus under general anaesthesia (GA) and
axillary block. A delto-pectoral approach was used to
expose the fracture. The bone ends were found to be
bleeding satisfactorily but there was no medullary canal.
Drill holes were inserted for a short distance. The cortex
adjacent to the fracture was petalled with osteotome. A 2.5
mm high-speed steel (HSS) drill bit (Synthes, UK) was
used with saline cooling. A drill motor with low speed and
high torque was used. The drill was frequently removed
from the bone to clear the flutes of dense accumulated
bone swarf (Figure 2) and saline irrigation was used at all
times. The holes were then over-drilled with a standard
drill bit to the required 3.2 mm to accept a 4.5 mm screw.
A standard 4.5 mm cortical tap was used, frequently
reversed and withdrawn for cleaning. A standard 3.2 mm
drill was used to attain the right diameter. A plate of suffi-
cient length was used to reach beyond the area of the stress
line. It was possible to achieve a secure hold with all the
screws. After reduction and fixation, Bone Morphogenic
Protein-7 (BMP-7) paste (OP1, Stryker, UK) was applied
around the fracture site before closure. The BMP Ossigraft
(OP1) was prepared and applied all around the fracture
site. A support sling was used for 6 weeks although limited
active assisted mobilisation was started on the second
postoperative day. After 3 months, there was good evi-
dence of callus formation and fracture healing (Figure 3)
along with a full range of motion at the shoulder joint.
Discussion
Currently, osteopetrosis is considered to be a syndrome
with excessive bone density occurring as a result of abnor-
mal function of osteoclasts [3]. Three clinically distinct
forms of osteopetrosis have been recognised – the infan-
tile malignant autosomal recessive form, the intermediate
autosomal recessive form and the adult benign autosomal
dominant form. The disease represents a spectrum of clin-
ical variants because of the heterogeneity of genetic
defects resulting in osteoclast dysfunction [4]. The pro-
pensity to fracture is seen in all three types but is a major
complication in the autosomal dominant form because of
the normal life span of patients in this category [5]. Most
of the fracture patterns are transverse or short oblique and
involve diaphyseal fractures of the long bones of the
upper and lower extremities. These can be managed suc-
cessfully non-operatively especially in children, however
time for healing is often prolonged [6,7]. Operative man-
agement of diaphyseal fractures is useful for patients
where the fractures are recalcitrant to conservative treat-
ment or where there is a risk of developing a disabling
deformity, such as with recurrent fractures or pre-existing
deformities [6]. Operative fracture management can be
technically difficult due to hard brittle bones without a
medullary canal. Re-fracture and infection of non-united
fractures have been reported after operative management,
particularly with screw plate fixation [8]. In order to over-
come the technical difficulties regarding drilling and
reaming of hard sclerotic bones, recommendations have
Pre-operative anterior-posterior/lateral view of non-united fractur  of the proximal humerus with osteopetr sisFigure 1
Pre-operative anterior-posterior/lateral view of non-
united fracture of the proximal humerus with osteo-
petrosis.
High-speed steel drill bit used for drilling osteopetrotic scle-rosed boneFi ure 2
High-speed steel drill bit used for drilling osteo-
petrotic sclerosed bone.Page 2 of 3
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Journal of Medical Case Reports 2009, 3:15 http://www.jmedicalcasereports.com/content/3/1/15been made to use high speed electric drill bits, frequently
cooling them and clearing the flutes while drilling and
using the graduated drill bit system to overcome drill
breakage and over-heating [9,10]. However, after internal
fixation, implant failure and non-union are still a major
risk [6,8]. We successfully overcame this complication by
using Bone Morphogenic Protein (BMP) which plays a
crucial role in bone formation by stimulating mesenchy-
mal cells and differentiating them into osteoblasts [11].
BMP has proved to be a very good tool because of its oste-
oinductive property, resulting in good callus formation
and healing of fractures.
Conclusion
Osteopetrosis introduces technical limitations to the tra-
ditional treatment of fracture management that may be
minimised with specific pre-operative planning. In the
treatment of non-united fractures in osteopetrosis, the use
of an HSS drill bit along with careful attention to drilling
technique can help avoid bit breakage and thermal bone
injury that may produce ring sequestrum or destroy the
already scant osteogenic cells. BMP-7 may be used as an
osteoinductive agent in this situation.
Abbreviations
BMP: bone morphogenic protein; HSS: high speed steel
Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
IR and DB conceived the study, participated in its design
and coordination and helped to draft the manuscript. JFH
and IR conducted the operation. JFH and AK revised the
article for intellectual content while IR and AK carried out
the literature review and the review of the patient's medi-
cal records. All authors read and approved the final man-
uscript.
References
1. Albers-Schonberg H: Rottgenbilder einer seltenen knochener-
krankung.  MMW Munch Med Wonchenschr 1904, 51:365.
2. Kocher MS, Kesser JR: Osteopetrosis.  Am J Orthop 2003,
32:222-228.
3. Shapiro F: Osteopetrosis: current clinical considerations.  Clin
Orthop 1993, 294:34-44.
4. Shapiro F, Glimcher MJ, Holtrop ME, Tashjian AH, Brickley-Parsons
D, Kenzora JE: Human osteopetrosis; a histological, ultrastruc-
tural and biochemical study.  J Bone Joint Surg Am 1980,
62:384-399.
5. Bollerslev J, Mosekilde L: Autosomal dominant osteopetrosis.
Clin Orthop 1993, 294:52-63.
6. Armstrong DG, Newfield JT, Gillespic R: Orthopedic manage-
ment of osteopetrosis: results and review of literature.  J Pedi-
atr Orthop 1999, 19:122-132.
7. Dahl N, Holmgren G, Holmberg S, Ersmark H: Fracture patterns
in malignant osteopetrosis (Albers-Schönberg disease).  Arch
Orthop Trauma Surg 1992, 111:121-123.
8. Milgram JW, Jasty M: Osteopetrosis: Morphological study of
twenty one cases.  J Bone Joint Surg Am 1982, 64:912-929.
9. Chhabra A, Westerland L, Kline AJ: Management of proximal
femoral shaft fractures in osteopetrosis: A case series using
internal fixation.  Orthopedics 2005, 28(6):587-592.
10. Strickland JP, Berry DJ: Total joint arthroplasty in patients with
osteopetrosis: A report of 5 cases and review of the litera-
ture.  J Arthroplasty 2005, 20(6):815-819.
11. Derner R, Anderson AC: The bone morphogenic protein.  Clin
Podiatr Med Surg 2005, 22:607-618.
Postoperative X-ray image of fracture after 6 months with healing and good reductionFigure 3
Postoperative X-ray image of fracture after 6 
months with healing and good reduction.Page 3 of 3
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