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

Case Report

iMedPub Journals Health Science Journal 2015 http://journals.imedpub.com ISSN 1791-809X Vol. 10 No. 1:12

Care of a Patient with Fibrous Dysplasia of the Agnieszka Kruk1, Janina Książek1, Clavicle and Ribs: Case Report Wojciech Żurek2, Sylwia Terech1, Piotr Jarzynkowski1 and Abstract Renata Piotrkowska1 Introduction: Fibrous dysplasia of the ribs and clavicle is a polyostotic form of 1 Department of Surgical Nursing, Medical skeletal system disease. The most commonly reported symptom is pain. Surgical University of Gdansk, Poland treatment, which is the primary therapeutic approach, may contribute to the 2 Faculty and Clinic of Chest Surgery, emergence of complications in surgical site that are difficult to treat. The aim of Medical University of Gdansk, PolandIran 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 Correspondence: Janina Książek 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. Results: Progressive disease process caused a lot of health problems in the patient  [email protected] and deterioration in the quality of life in every aspects of his life . Conclusions and relevance for clinical practice: Care and treatment in hospital Zakład Pielęgniarstwa Chirurgicznego, and at home are focused on the pain and chronic wound healing process, which Katedra Pielęgniarstwa GUMed, Dębinki 7, require the involvement of the entire interdisciplinary team. 15, Poland.

Keywords: Fibrous dysplasia of bone; Smoking; Interdisciplinary care; Chronic Tel: +48 58 3491247 wound healing; Education Citation: Kruk A, Książek J, Żurek W, et al., Care of a Patient with Fibrous Dysplasia of the Received: November 30, 2015; Accepted: December 12, 2015; Published: December Clavicle and Ribs: Case Report. Health Sci J. 30, 2015 2015, 10:1.

Introduction Epidemiological data say that it constitutes 2.5% of tumor-like changes and about 7% of benign tumors [3,4]. Fibrous dysplasia - FD (lat. dysplasia fibrosa – DF) is a rare The World Health Organization defines three forms of the chronic bone disease, characterized by the presence of one or disease: 1) monostotic, which accounts for about 70% of all more areas where healthy bone is replaced by fibrous tissue [1]. cases and affects one bone; 2) polyostotic, when the changes are The cause of this disease is not fully understood. It is currently observed in many bones; and 3) the form in which the disease considered that the reason for its development is a mutation involves craniofacial bones only. McCune-Albright syndrome in the GNAS1 gene, which leads to the production of abnormal is a polyostotic form where, in addition to multifocal changes, G protein responsible for the regulation of bone synthesis. This disorders of the endocrine glands occur (precocious puberty, promotes the development of metabolic bone disorders. Normal hyperthyroidism, hyperpituitarism, hyperadrenocorticism, trabecular bone structure is rebuilt in fibrous tissue, in which, overactive adrenal medulla) and the presence of café au lait as the disease progresses, metaplastic bone structure occurs. spots on the skin, scoliosis [1,4]. The image of the disease may This can cause impaired growth, brittleness, and deformation of be preceded by the appearance of disease symptoms, such as bones affected by disease process. The effects of mutation apply pain, deformations, and bone fractures. However, the diagnosis also to endocrine balance and disrupt the body pigmentation [2]. is based on the result of histopathological and imaging tests. The clinical picture of a generalized form of FD (lat. osteitis fibrosa An additional diagnostic procedure is the determination of the alkaline phosphatase level in serum. generalisata) was first described in 1981 by Recklinghausen. It was only in 1938 when Lichtenstein distinguished fibrous dysplasia of The treatment of choise is surgery if possible, which reduces pain bone as a separate disease entity (lat. dysplasia fibrosa ossium) and improves mobility, and a secondary healing of fractures [5]. [1,2]. The incidence of the disease is not precisely known. The surgery involves the removal of pathological focus with a

margin of healthy bone and subsequent tissue transplantation. In plexus at the left side was performed. The image clearly showed some cases a stabilizing action is required with the use of metal compression of the upper lobe of the left lung by pathological implants and bone cement. Conservative treatment consists tissue with low signal at time T1 and T2 dependent. Probably the of regularly taking bisphosphonates, which influence bone changes corresponded to the postoperative scar and partly to remodeling, reduce pain, and slow the pathogenic processes. the main process - FD. Nerve trunks with blurred outlines were Availability of bisphosphonates in Poland is limited because they seen, probably in the course of accompanying fibrosis due to have not been registered for the treatment of FD [6,7]. compressive and postoperative changes. What does this Paper contribute to the wider In 1999, on the basis of radiological documentation from the years global clinical community? 1994 to 1999, an opinion was given, based on which the constant progression of lesions of the dysplasia fibrosa ossium type was The described case shows the need for the introduction of stated. The nature of progression was uneven, i.e. on the right consultation meetings involving specialist doctors, psychologists, side it is clearly slower than on the left side, where currently the and health workers into clinical practice in the care of FD patients. morphological status of the bone corresponds almost exactly to Methods the degree of severity of the changes just before surgery. In 2001, the patient was again qualified for surgery, in this time The interview, observation, analysis of medical records, and case for partial resection of the left clavicle. In anatomopathological study method was used in the study. The place of research is the Department of Thoracic Surgery, University Clinical Center, examination of surgical specimen irregular trabeculae of bone Medical University of Gdansk. The case describes a 51-year-old and intertrabecular spaces filled with the connective tissue man diagnosed with fibrous dysplasia of the clavicle and ribs, in larger spaces in the purulent inflammation of the clavicle hospitalized in the years 1995-2013. The report is additionally fragment were seen. There were no characteristics of fibrous supplemented with figures of the wound healing process in years dysplasia of bone. 2012-2014. In 2004, resection of a fragment of the sternum, part of clavicle, and The aim of this case report is to analyze interdisciplinary care of first and second rib on the left was performed, and subsequently a patient chronically treated for fibrous dysplasia of the clavicle the patient was qualified for chest radiation in a single fraction and ribs . 6Gy. Following treatment the patient was discharged home. In 2006, the patient came to the Department of Thoracic Surgery Case Report because of severe pain. It was caused by compression of the A 51-year-old male, heavy smoker (30 pack years, without hypertrophic clavicle and first rib on the brachial plexus on the willingness to quit the habit) was treated for bone fibrous dysplasia right side. During qualification for the surgery, the patient was since 1994. The first symptoms appeared at the age of 30. The found to be a carrier of hepatitis C. Right clavicle resection and patient came to the physician because of severe pain in the chest. partial resection of the first rib were done. The postoperative In 1995, based on computed tomography scan (CT) of the chest, course was uneventful. The patient was discharged home with 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 Figure 1 3D CT reconstruction of bony elements of the chest for a month, then a month break, was used for the next three after resection of upper ribs and upper part of the years. Despite therapy, the man continued to suffer pain. sternum. Source: Own material provided by the patient. In 1998, an Magnetic Resonance Imaging (MRI) of the brachial

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instructions for urgent control in the Pain Clinic to reduce the the hospital with purulent fistula at the upper part of the sternum, doses of opioid drugs. The macroscopic test result confirmed the for further treatment. Revision of the wound in the upper part clinical diagnosis corresponding to FD. of the sternum was performed under general anesthesia. In that time patient reported progressive weakness with body weight On the CT image from 2007, bone distension and deformation loss of approximately 10% of his total body weight. Now he is a was visible; discreetly uneven internal profile of the cortex with lonely person, living from social welfare benefits. He is under the small foci of sclerosis (Figure 1). care of a pain clinic, and he is addicted to narcotic drugs for pain. Because of the persistent chest pain and visible changes in the He currently takes medication in doses: Morphine 40 mg (s.c.) radiographic image in 2008, surgical treatment was continued. 4 x daily; MST 200 Continus 200 mg/ BD Sevredol 20 mg/tablet The sternum and a fragment of the chest wall were resected with 4 x daily; and Ketoprofene 50 mg/tablet 4 x daily. Additionally, mesh implantation. Resection of the second and third rib on the Diazepam 5 mg/tablet 2-3 times a day, and due to difficulty in right side was performed. A year later, the patient came to the falling asleep Lorazepam 2.5 mg/tablet once a day were included. hospital because of purulent fistula in the postoperative wound. He requires the assistance of another person in the field of wound Plastic surgery of the chest wall was performed and antibiotic care (Figures 3 and 4) and remains under the ambulatory care of therapy included. The patient was discharged on the seventh a surgeon and a health worker. Table 1 shows the course of the postoperative day. In 2009, the patient was hospitalized again for therapy and nursing care over the years 1995 to 2013. recurrent skin-mediastinal fistula. The fistula was excised with skin-muscle flap plastic reconstruction was performed. In 2010, Discussion the patient was hospitalized twice because of chronic suppuration Surgical treatment of FD is a complex process with uncertain of the wound. Revision of the wound was performed. The skin- results and often requires repeated operations. At a time when muscle fistula was excised with wound left for granulation In 2011 significant attention is devoted worldwide to stem cells asa an improvement in the wound healing process was achieved, and potential tool for curing incurable diseases, fibrous dysplasia of the decision to close it was made. In the further postoperative bone (FD) provides a paradigm for stem cell diseases. Recognizing course the patient developed symptoms of inflammation again, the wound was opened, and treatment with the use of dressings FD as a stem cell disease gives way to generate appropriate models was continued. The bacteriological test confirmed methicillin- of the disease, which will continue to provide further insight into resistant Staphylococcus aureus (MRSA) wound infection. its natural history and pathogenesis. It is believed that skeletal During hospitalization the patient was treated with the vacuum stem cells may be a tool for innovative treatments. These can be dressing.. After achieving local improvement and after consulting conceived as directed to alter the in vivo behavior of mutated the plastic surgeon the patient was qualified for surgery. The stem cells, to replace mutated cells through local transplantation, granulating chest wound was covered with a skin autograft from or to correct the genetic defect in the stem cells themselves. In the left thigh. In the following postoperative days the wound was vitro and in vivo models are currently being generated and results without signs of inflammation. The patient was discharged home. are being expected [2]. In 2012, outpatient therapy was needed because of wound Several years of experience with treatment and nursing care suppuration (Figure 2). In September 2013, the patient came to 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 torefer 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 Picture of the chest wound around the upper part of the Figure 2 studies have shown the existence of genetic predisposition to sternum, November 2012. Prolongated healing of the wound after full thicknes skin transplantation with areas addiction, either to drugs or other psychoactive substances of granulation. Prolongated healing due to local infection [11,12]. with discharge and scabbing. Treatment of chronic wound is a difficult and long-term process Source: own material. that requires close cooperation between specialists. In this case, there were a chest surgeon, primary care physician, nurses © Under License of Creative Commons Attribution 3.0 License 3 ARCHIVOSHealth Science DE MEDICINA Journal 2015 ISSNISSN 1698-94651791-809X Vol. 10 No. 1:12

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 1. Education in wound care and overcoming pain in the home Figure 3 Picture of the chest wound around the upper part of environment requires constant support of physicians and the sternum, June 2013. Healing of the wound after full nurses and special attention paid to the patient's psychosocial thicknes skin transplantation with still visible areas of requirements. granulation without epithelisation (oozing granulation). Light pink colour cicatrix in the process of remodelling 2. Chronic smoking indicates a need to focusing on the problem with more smooth surface. of health behaviors and their impact on wound healing, and Source: own material. 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 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 Figure 4 Picture of the chest wound around the upper part of the epidemiological indicators, previously used in the treatment sternum, January 2014. process, became insufficient for evaluation of some diseases, The scar in majority is remodelled with light, smooth especially chronic ones. surface. There is overgrown granulation on the right side The described case shows the need for the introduction of of the scar. Source: own material. multidisciplinary consultation meetings involving specialist doctors, psychologists, and health workers into clinical practice in the care of FD patients. working in the surgical department, and a health visitor who takes care of patients in their home. Nurses and physicians must Conflict of Interest continuously update their knowledge in the field of new wound None of the authors declared any conflict of interest. treatment regimens to ensure the effective treatment for the patient in accordance with the current treatment algorithm. Acknowledgements A vacuum-based method of wound healing was applied during the patient's stay in hospital, which resulted in the desired The language assistance was provided by Proper Medical Writing therapeutic effect. The patient was discharged home with Sp. z o.o., Warsaw, Poland. instructions to continue the treatment of wounds under the

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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 Diagnosis and treatment Selected problems of nursing care • The emergence of severe chest pain. • Anxiety caused by the onset of 1994, 1995 UCK • 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. 1995 • Resection of parasternal fragments of ribs II-III on the left side. • Knowledge deficit of the surgical PCT & UCK • Histopathological examination revealed fibrous dysplasia of clavicle site care. and ribs I, II, and III. • Fear as a result of the diagnosis of FD. 2001 PCT & • Excision of dysplasia focus in the proximal left clavicle. • Emotional problems as a result of 2002 OSK • Partial resection of the right clavicle. marriage breakdwn. • Resection of the fragment of sternum, clavicle, 2004 PCT • 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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