Download Care of a Patient with Fibrous Dysplasia of the Clavicle and Ribs

Case Report
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Care of a Patient with Fibrous Dysplasia of the
Clavicle and Ribs: Case Report
Abstract
Introduction: Fibrous dysplasia of the ribs and clavicle is a polyostotic form of
skeletal system disease. The most commonly reported symptom is pain. Surgical
treatment, which is the primary therapeutic approach, may contribute to the
emergence of complications in surgical site that are difficult to treat. The aim of
the study was to analyze interdisciplinary care of a patient chronically treated for
fibrous dysplasia of the clavicle and ribs.
Methods: The interview, observation, and analysis of medical records method was
used in the study. The case describes a 51-year-old man with a diagnosis of fibrous
dysplasia of the clavicle and ribs, hospitalized in the Department of Thoracic
Surgery of Medical University of Gdansk (MUG) in the years 1995-2013.
2015
Vol. 10 No. 1:12
Agnieszka Kruk1,
Janina Książek1,
Wojciech Żurek2,
Sylwia Terech1,
Piotr Jarzynkowski1 and
Renata Piotrkowska1
1 Department of Surgical Nursing, Medical
University of Gdansk, Poland
2 Faculty and Clinic of Chest Surgery,
Medical University of Gdansk, PolandIran
Correspondence: Janina Książek
Results: Progressive disease process caused a lot of health problems in the patient
and deterioration in the quality of life in every aspects of his life .

Conclusions and relevance for clinical practice: Care and treatment in hospital
and at home are focused on the pain and chronic wound healing process, which
require the involvement of the entire interdisciplinary team.
Zakład Pielęgniarstwa Chirurgicznego,
Katedra Pielęgniarstwa GUMed, Dębinki 7,
15, Poland.
Keywords: Fibrous dysplasia of bone; Smoking; Interdisciplinary care; Chronic
wound healing; Education
Tel: +48 58 3491247
Received: November 30, 2015; Accepted: December 12, 2015; Published: December
30, 2015
Introduction
Fibrous dysplasia - FD (lat. dysplasia fibrosa – DF) is a rare
chronic bone disease, characterized by the presence of one or
more areas where healthy bone is replaced by fibrous tissue [1].
The cause of this disease is not fully understood. It is currently
considered that the reason for its development is a mutation
in the GNAS1 gene, which leads to the production of abnormal
G protein responsible for the regulation of bone synthesis. This
promotes the development of metabolic bone disorders. Normal
trabecular bone structure is rebuilt in fibrous tissue, in which,
as the disease progresses, metaplastic bone structure occurs.
This can cause impaired growth, brittleness, and deformation of
bones affected by disease process. The effects of mutation apply
also to endocrine balance and disrupt the body pigmentation [2].
The clinical picture of a generalized form of FD (lat. osteitis fibrosa
generalisata) was first described in 1981 by Recklinghausen. It was
only in 1938 when Lichtenstein distinguished fibrous dysplasia of
bone as a separate disease entity (lat. dysplasia fibrosa ossium)
[1,2]. The incidence of the disease is not precisely known.
© Copyright iMedPub | This article is available in: www.hsj.gr/archive
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Citation: Kruk A, Książek J, Żurek W, et al.,
Care of a Patient with Fibrous Dysplasia of the
Clavicle and Ribs: Case Report. Health Sci J.
2015, 10:1.
Epidemiological data say that it constitutes 2.5% of tumor-like
changes and about 7% of benign tumors [3,4].
The World Health Organization defines three forms of the
disease: 1) monostotic, which accounts for about 70% of all
cases and affects one bone; 2) polyostotic, when the changes are
observed in many bones; and 3) the form in which the disease
involves craniofacial bones only. McCune-Albright syndrome
is a polyostotic form where, in addition to multifocal changes,
disorders of the endocrine glands occur (precocious puberty,
hyperthyroidism,
hyperpituitarism,
hyperadrenocorticism,
overactive adrenal medulla) and the presence of café au lait
spots on the skin, scoliosis [1,4]. The image of the disease may
be preceded by the appearance of disease symptoms, such as
pain, deformations, and bone fractures. However, the diagnosis
is based on the result of histopathological and imaging tests.
An additional diagnostic procedure is the determination of the
alkaline phosphatase level in serum.
The treatment of choise is surgery if possible, which reduces pain
and improves mobility, and a secondary healing of fractures [5].
The surgery involves the removal of pathological focus with a
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margin of healthy bone and subsequent tissue transplantation. In
some cases a stabilizing action is required with the use of metal
implants and bone cement. Conservative treatment consists
of regularly taking bisphosphonates, which influence bone
remodeling, reduce pain, and slow the pathogenic processes.
Availability of bisphosphonates in Poland is limited because they
have not been registered for the treatment of FD [6,7].
plexus at the left side was performed. The image clearly showed
compression of the upper lobe of the left lung by pathological
tissue with low signal at time T1 and T2 dependent. Probably the
changes corresponded to the postoperative scar and partly to
the main process - FD. Nerve trunks with blurred outlines were
seen, probably in the course of accompanying fibrosis due to
compressive and postoperative changes.
What does this Paper contribute to the wider
global clinical community?
In 1999, on the basis of radiological documentation from the years
1994 to 1999, an opinion was given, based on which the constant
progression of lesions of the dysplasia fibrosa ossium type was
stated. The nature of progression was uneven, i.e. on the right
side it is clearly slower than on the left side, where currently the
morphological status of the bone corresponds almost exactly to
the degree of severity of the changes just before surgery.
The described case shows the need for the introduction of
consultation meetings involving specialist doctors, psychologists,
and health workers into clinical practice in the care of FD patients.
Methods
The interview, observation, analysis of medical records, and case
study method was used in the study. The place of research is
the Department of Thoracic Surgery, University Clinical Center,
Medical University of Gdansk. The case describes a 51-year-old
man diagnosed with fibrous dysplasia of the clavicle and ribs,
hospitalized in the years 1995-2013. The report is additionally
supplemented with figures of the wound healing process in years
2012-2014.
The aim of this case report is to analyze interdisciplinary care of
a patient chronically treated for fibrous dysplasia of the clavicle
and ribs .
Case Report
A 51-year-old male, heavy smoker (30 pack years, without
willingness to quit the habit) was treated for bone fibrous dysplasia
since 1994. The first symptoms appeared at the age of 30. The
patient came to the physician because of severe pain in the chest.
In 1995, based on computed tomography scan (CT) of the chest,
features of thickening of the proximal parts of clavicles and ribs I
and II on the left side in the parasternal part with characteristics
of fibrous-like intraosseous hypertrophy and sclerosis. In addition
to intraosseous changes, thickening of the adjacent soft tissues
was observed. In the CT image bone remodeling characteristic
for fibrous dysplasia of the clavicle and ribs was observed.
Initially, the patient was qualified for the partial resection of the
left clavicle. After three months thoracotomy was done with
resection of parasternal fragments of the second and the third rib
on the left. On the anatomopathological examination, the image
of FD was confirmed. Results of laboratory tests showed normal
serum levels of inorganic phosphorus, total calcium, and alkaline
phosphatase.
In 1996 the case was consulted by representatives of the Polish
Osteoporosis Foundation (Polska Fundacja Osteoporozy, PFO).
The PFO advised that due to the lack of established methods for
the treatment of FD, pharmacological treatment is recommended.
It was stated that the use of calcitonin, a known stimulant of
somatostatin should have a positive effect on bone morphological
image. According to the recommendations, treatment with
Miacalcic Nasal Spray, a 100 I.U. in a scheme of two times a day
for a month, then a month break, was used for the next three
years. Despite therapy, the man continued to suffer pain.
In 1998, an Magnetic Resonance Imaging (MRI) of the brachial
2
In 2001, the patient was again qualified for surgery, in this time
for partial resection of the left clavicle. In anatomopathological
examination of surgical specimen irregular trabeculae of bone
and intertrabecular spaces filled with the connective tissue
in larger spaces in the purulent inflammation of the clavicle
fragment were seen. There were no characteristics of fibrous
dysplasia of bone.
In 2004, resection of a fragment of the sternum, part of clavicle, and
first and second rib on the left was performed, and subsequently
the patient was qualified for chest radiation in a single fraction
6Gy. Following treatment the patient was discharged home.
In 2006, the patient came to the Department of Thoracic Surgery
because of severe pain. It was caused by compression of the
hypertrophic clavicle and first rib on the brachial plexus on the
right side. During qualification for the surgery, the patient was
found to be a carrier of hepatitis C. Right clavicle resection and
partial resection of the first rib were done. The postoperative
course was uneventful. The patient was discharged home with
Figure 1 3D CT reconstruction of bony elements of the chest
after resection of upper ribs and upper part of the
sternum.
Source: Own material provided by the patient.
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instructions for urgent control in the Pain Clinic to reduce the
doses of opioid drugs. The macroscopic test result confirmed the
clinical diagnosis corresponding to FD.
On the CT image from 2007, bone distension and deformation
was visible; discreetly uneven internal profile of the cortex with
small foci of sclerosis (Figure 1).
Because of the persistent chest pain and visible changes in the
radiographic image in 2008, surgical treatment was continued.
The sternum and a fragment of the chest wall were resected with
mesh implantation. Resection of the second and third rib on the
right side was performed. A year later, the patient came to the
hospital because of purulent fistula in the postoperative wound.
Plastic surgery of the chest wall was performed and antibiotic
therapy included. The patient was discharged on the seventh
postoperative day. In 2009, the patient was hospitalized again for
recurrent skin-mediastinal fistula. The fistula was excised with
skin-muscle flap plastic reconstruction was performed. In 2010,
the patient was hospitalized twice because of chronic suppuration
of the wound. Revision of the wound was performed. The skinmuscle fistula was excised with wound left for granulation In 2011
an improvement in the wound healing process was achieved, and
the decision to close it was made. In the further postoperative
course the patient developed symptoms of inflammation again,
the wound was opened, and treatment with the use of dressings
was continued. The bacteriological test confirmed methicillinresistant Staphylococcus aureus (MRSA) wound infection.
During hospitalization the patient was treated with the vacuum
dressing.. After achieving local improvement and after consulting
the plastic surgeon the patient was qualified for surgery. The
granulating chest wound was covered with a skin autograft from
the left thigh. In the following postoperative days the wound was
without signs of inflammation. The patient was discharged home.
In 2012, outpatient therapy was needed because of wound
suppuration (Figure 2). In September 2013, the patient came to
Figure 2 Picture of the chest wound around the upper part of the
sternum, November 2012. Prolongated healing of the
wound after full thicknes skin transplantation with areas
of granulation. Prolongated healing due to local infection
with discharge and scabbing.
Source: own material.
© Under License of Creative Commons Attribution 3.0 License
2015
Vol. 10 No. 1:12
the hospital with purulent fistula at the upper part of the sternum,
for further treatment. Revision of the wound in the upper part
of the sternum was performed under general anesthesia. In that
time patient reported progressive weakness with body weight
loss of approximately 10% of his total body weight. Now he is a
lonely person, living from social welfare benefits. He is under the
care of a pain clinic, and he is addicted to narcotic drugs for pain.
He currently takes medication in doses: Morphine 40 mg (s.c.)
4 x daily; MST 200 Continus 200 mg/ BD Sevredol 20 mg/tablet
4 x daily; and Ketoprofene 50 mg/tablet 4 x daily. Additionally,
Diazepam 5 mg/tablet 2-3 times a day, and due to difficulty in
falling asleep Lorazepam 2.5 mg/tablet once a day were included.
He requires the assistance of another person in the field of wound
care (Figures 3 and 4) and remains under the ambulatory care of
a surgeon and a health worker. Table 1 shows the course of the
therapy and nursing care over the years 1995 to 2013.
Discussion
Surgical treatment of FD is a complex process with uncertain
results and often requires repeated operations. At a time when
significant attention is devoted worldwide to stem cells as a
potential tool for curing incurable diseases, fibrous dysplasia of
bone (FD) provides a paradigm for stem cell diseases. Recognizing
FD as a stem cell disease gives way to generate appropriate models
of the disease, which will continue to provide further insight into
its natural history and pathogenesis. It is believed that skeletal
stem cells may be a tool for innovative treatments. These can be
conceived as directed to alter the in vivo behavior of mutated
stem cells, to replace mutated cells through local transplantation,
or to correct the genetic defect in the stem cells themselves. In
vitro and in vivo models are currently being generated and results
are being expected [2].
Several years of experience with treatment and nursing care
of patients with fibrous dysplasia of the clavicle and ribs
have revealed how serious and complex problem is for the
interdisciplinary team in the care of chronically treated patients.
Long-term surgical treatment of patients causes frequent changes
of the environment and can be a source of emotional tension,
leading even to social isolation.
Pain is one of the most frequent symptoms of fibrous dysplasia
(FD). The severity of pain in patients with FD is highly variable,
and it is more common in adults than in children. It has been
observed in 81% of adults and 49% of children [8,9,10]. Analysis
of the course of treatment shows that bone pain was the most
frequently reported symptom (Table 1). Basic analgesics have
insufficient effect, and such a situation leads doctors to refer
patients to specialist outpatient treatment of chronic pain. In the
case described, the patient has taken opioids for several years,
and their doses had to be modified during therapy due to the
increasing tolerance. In the group of patients treated chronically
with opioids, cases of addiction are reported sporadically, and
studies have shown the existence of genetic predisposition to
addiction, either to drugs or other psychoactive substances
[11,12].
Treatment of chronic wound is a difficult and long-term process
that requires close cooperation between specialists. In this case,
there were a chest surgeon, primary care physician, nurses
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control of a primary care physician and health visitor. The analysis
of this case revealed deterioration in wound healing during this
period of treatment (Figures 3 and 4). Outpatient care is primarily
focused on continuing education in the field of wound care and
health behaviors. The patient refuses to quit smoking despite
the fact that he has been a smoker for 30 years. Any attempts of
anti-smoking education during hospitalization did not bring the
expected result. The patient was informed about the negative
effects of smoking on wound healing [13].
In the last years of hospitalization increased somatic symptoms
and impaired functioning in many spheres of life were observed
in this patient. They can cause deterioration of quality of his life.
Studies previously published by Kelly, concerning the quality
of life in adults and children with FD, showed worse physical
functioning than in the control group [14].
Conclusions
Figure 3 Picture of the chest wound around the upper part of
the sternum, June 2013. Healing of the wound after full
thicknes skin transplantation with still visible areas of
granulation without epithelisation (oozing granulation).
Light pink colour cicatrix in the process of remodelling
with more smooth surface.
Source: own material.
1. Education in wound care and overcoming pain in the home
environment requires constant support of physicians and
nurses and special attention paid to the patient's psychosocial
requirements.
2. Chronic smoking indicates a need to focusing on the problem
of health behaviors and their impact on wound healing, and
consideration of professional anti-smoking therapy for the
patient.
3. Long-term results of surgical treatment of multifocal form
of FD are uncertain and the treatment is long lasting. The
chronicity of the treatment significantly affects the psyche of
the patient and requires psychological support.
4. Steam cell therapy is possible promising approach as a new
FD therapy directed to alter the in vivo behavior of mutated
stem cells – in vitro and in vivo models are currently being
generated and final results are expected.
5. Treatment of FD patients should be carefully planned by
multidisciplinary consultation meetings with the participation
of physicians, psychologists, and health worker.
Relevance for Clinical Practice
Figure 4 Picture of the chest wound around the upper part of the
sternum, January 2014.
The scar in majority is remodelled with light, smooth
surface. There is overgrown granulation on the right side
of the scar.
Source: own material.
working in the surgical department, and a health visitor who
takes care of patients in their home. Nurses and physicians must
continuously update their knowledge in the field of new wound
treatment regimens to ensure the effective treatment for the
patient in accordance with the current treatment algorithm.
A vacuum-based method of wound healing was applied during
the patient's stay in hospital, which resulted in the desired
therapeutic effect. The patient was discharged home with
instructions to continue the treatment of wounds under the
4
The aim of the study is to draw attention to fibrous dysplasia of
the clavicle and ribs, uncommon in clinical practice. Previously
published works that describe the clinical image of the disease
often ignore issues related to the functioning of the patient and
the influence of the treatment on quality of life. Physical and
epidemiological indicators, previously used in the treatment
process, became insufficient for evaluation of some diseases,
especially chronic ones.
The described case shows the need for the introduction of
multidisciplinary consultation meetings involving specialist
doctors, psychologists, and health workers into clinical practice in
the care of FD patients.
Conflict of Interest
None of the authors declared any conflict of interest.
Acknowledgements
The language assistance was provided by Proper Medical Writing
Sp. z o.o., Warsaw, Poland.
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Table 1. The course of the disease with regard to surgical methods for the treatment and care of selected problems.
Years of hospitalization
1994, 1995 UCK
1995
PCT & UCK
2001 PCT &
2002 OSK
2004 PCT
Diagnosis and treatment
Selected problems of nursing care
•The emergence of severe chest pain.
•Anxiety caused by the onset of
•Outpatient treatment. The X-ray examination of the chest disease symptoms.
thickening of the clavicle bones and ribs on the left side was found.
•Anxiety caused by
•The resection of the fragment of the clavicle on the left side.
prolonged diagnostic process.
•Resection of parasternal fragments of ribs II-III on the left side.
•Knowledge deficit of the surgical
•Histopathological examination revealed fibrous dysplasia of clavicle site care.
and ribs I, II, and III.
•Fear as a result of the diagnosis
of FD.
•Excision of dysplasia focus in the proximal left clavicle.
•Emotional problems as a result of
•Partial resection of the right clavicle.
marriage breakdwn.
•Resection of the fragment of sternum, clavicle,
• rib I and II on the left side.
•Knowledge deficit due to the
•Teletherapy - Cliniac linear accelerator 6 MeV photons on the chest
inclusion of a new therapy.
2004 UCK
area with the technique of 2 parallel opposed fields of dimensions
•The risk of complications after
11x15 cm Dgref. = 6Gy/1FR.
teletherapy.
•Removal of the right clavicle and partial resection of rib I on the
•Chronic pain.
right side.
2006 UCK
•Possibility of complications
•Consultation in chronic pain clinic. Suspected addiction to
resulting from smoking habit.
analgesics.
•The intensification of pain and radiological changes of the chest.
•Resection of the clavicle and the fragment of the chest wall with
•Anxiety caused by chronic wound
2008 UCK
mesh implantation.
treatment.
•Resection of ribs II and III on the right side.
•Disorders in the correct health
•Postoperative wound suppuration.
2008 UCK
behaviors.
•Thoracomioplastic operation.
•Anti-smoking education
•Treatment for chronic skin/mediastinal fistula.
2009 UCK
•Resection of fistula and skin/muscle plastic surgery
•Resection of skin/mediastinal fistula
2010 UCK
•Wound revision
•Plastic surgery and postoperative wound closure.
•Knowledge deficit of the care of
•In the course of postoperative care the wound was opened and
surgical site.
2011 UCK
treatment was continued with the use of the vacuum based
•Discomfort due to chronic
dressings method.
infection of the wound.
•Fear as a result of the diagnosis of FD.
2012
•Outpatient treatment
•The progressive body weight loss.
•Difficult financial situation.
2013 Specialist Hospital •Surgery of suppurative fistula at the upper part of the sternum.
•Loneliness.
in Prabuty
•Resection of the mesh and the chest wall.
•Anxiety caused by chronic wound
treatment.
Source: author’s own analysis.
Description: 1. UCK: University Clinical Centre in Gdansk, 2. PCT: Pomeranian Centre of Traumatology in Gdansk. (Copernicus), 3.
OSK: District Railway Hospital in Gdansk.
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References
1 Dixon JL, Smythe WR, Rascoe PA, Reznik SI (2013) Surgical resection
of giant fibrous dysplasia for near respiratory collapse. Ann Thorac
Surg 95: 135-137.
2 Riminucci M, Saggio I, Robey PG, Bianco P (2006) Fibrous dysplasia as
a stem cell disease. J Bone Miner Res 21: 125-131.
3 Mohan H, Mittal P, Mundi I, Kumar S (2011) Fibrous dysplasia of bone: A
clinicopathologic review. Pathol Lab Med Int 3: 31-42.
4 Bhadada S, Bhansali A, Das S, Singh R, Sen R, et al (2011) Fibrous
dysplasia & McCune-Albright syndrome: An experience from a
tertiary care centre in north India. Indian J Med Res 133: 504-509.
5 Ozkaya S, Findik S, Demir H, Yuksel C, Atici AG (2009) Polyostotic
fibrous dysplasia of the ribs: An unusual cause of chest pain and
dyspnea. Int Med Case Rep J 2: 23-25.
2015
Vol. 10 No. 1:12
8 Weerasuriya T, Nashi S, Morgan A, Ebizie A (2012) A dilemma of
fibrous dysplasia versus chronic osteomyelitis of the clavicle. BMJ
Case Rep 2012.
9 Chapurlat RD, Gensburger D, Jimenez-Andrade JM, Ghilardi JR, Kelly
M, et al. (2012) Pathophysiology and medical treatment of pain in
fibrous dysplasia of bone. Orphanet J Rare Dis 7 Suppl 1: S3.
10 Kelly MH, Brillante B, Collins MT (2008) Pain in fibrous dysplasia
of bone: age-related changes and the anatomical distribution of
skeletal lesions. Osteoporos Int 19: 57-63.
11 Jimenez-Andrade JM, Mantyh WG, Bloom AP, Freeman KT, Ghilardi
JR, et al. (2012) The effect of aging on the density of the sensory
nerve fiber innervation of bone and acute skeletal pain. Neurobiol
Aging. 33: 921-932.
12 Clark JD (2002) Chronic pain prevalence and analgesic prescribing in
a general medical population. J Pain Symptom Manage 23: 131-137.
6 DiCaprio MR, Enneking WF (2005) Fibrous dysplasia. Pathophysiology,
evaluation, and treatment. J Bone Joint Surg Am 87: 1848-1864.
13 Sorensen L (2012) Wound healing and infection in surgery. The
clinical impact of smoking and smoking cessation: A systematic
review and meta-analysis. Arch Surg 147: 373-383.
7 Stanton RP, Ippolito E, Springfield D, Lindaman L, Wientroub S, et
al. (2012) The surgical management of fibrous dysplasia of bone.
Orphanet J Rare Dis 7 Suppl 1: S1.
14 Kelly MH, Brillante B, Kushner H, Robey GP, Collins MT (2005)
Physical function is impaired but quality of life preserved in patients
with fibrous dysplasia of bone. Bone 37: 388-394.
6
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