23. Multi-site avascular necrosis of idiopathic aetiology

Abstract

Introduction: Avascular necrosis is defined as the death of bone tissues secondary to lack of inadequate vascular supply. Its aetiology is numerous, and sometimes multifactorial, but in essence may be caused by any perturbation in bony vasculature 1. Such examples range from fracture, joint dislocation, slipped and epiphysis, to more complex diseases such as sickle cell anaemia, rheumatoid arthritis and systemic lupus erythematosus. Furthermore, exogenous causes such as alcohol abuse or corticosteroid administration 1,2 are also apparent, although with a caveat that in 25% of cases the cause remains unknown. All in all, this in turn leads to bone necrosis, which subsequently may lead to osteochondritis dissecans, should articular joint cartilage become involved. The most common affected sites are the following 3: 1) head of femur; 2) neck of talus or 3) scaphoid 1,2. After the bodily insult, anoxia of haematopoietic cells causes bony cell death within 12-48 hours, with bone marrow fat cell death within 5 days. Case description: Herein we present an interesting case of a 41 year old male accountant, of origin from Tenerife. This gentleman presented as a GP referral to our outpatient rheumatology clinic earlier this year with right sided hip pain and general polyarthralgia. On direct questioning, he was afflicted with this for the prior 3-4 years, affecting his knees, ankles, wrists, fingers and neck. He furthermore disclosed morning stiffness and difficulty in long distance mobility, but no joint swelling. He was taking over the counter analgesia only, to minimal avail, and was a never-smoker, non-drinker. Serologically, his inflammatory markers were negative, and all rheumatological screen was negative. Screening for haemaglobinopathy, myeloma, infection and bone profile were all normal/within normal range limits. On examination, these gentleman exhibited limited range-of-movement (ROM) to his ankles bilaterally, with tenderness. He furthermore displayed tenderness to knee palpation with mild crepitus, albeit to a full ROM. His right hip was particularly tender, with limited ROM, as was his left wrist and chest wall. With this gentleman’s clinical findings, he was subsequently scheduled for X-Ray imaging of his left wrist, ankles (bilaterally) and hip. Strikingly, the X-ray imaging revealed avascular necrosis to the following: 1) left scaphoid, 2) talus/calcaneum bilaterally and 3) femoral head, also bilaterally. Thus, this gentleman presented with all 3 of the top three sites for avascular necrosis. For medical management, he was subsequently given NSAID/further analgesia, zolendronic acid infusion (to no improvement) and referred to the orthopaedic team for consideration of joint replacement/resurfacing. Discussion: Avascular necrosis typically affects one joint in a given individual for a presenting complaint. However, in this interesting case we present a young gentleman with 5 affected joints to avascular necrosis, all of which engendering the ‘top three’ most common sites . It is typically diagnosed by X-Ray, MRI with or without bone scan, and treatments are wide and encompass firstly medical, such as with the use of non-steroidal anti-inflammatory drugs (NSAIDS), bisphosphonates and secondary prevention with statins/anticoagulation as required 3,4. Following which, surgical options are also apparent, and include core decompression, bone grafting, osteotomy or arthroplasty. Although this patient did not respond to zolendronic acid infusion, he is now under consideration for prospective iloprost infusions 5. Key learning points: Avascular necrosis has a myriad of causes, although in excess of 25% of cases they remain idiopathic. The most common sites affected are that of the femur, scaphoid and ankle, with investigations to confirm this dependant on radiology. Management comprises that of combined therapy with physiotherapy, medical management, with consideration to surgical intervention.

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she continued with the sport before having to give it up completely due to
her symptoms. She also developed sciatica symptoms 10 months ago, in
additiontoher arthralgia for which she sought treatment from a chiroprac-
tor, which unfortunately provided no relief. In terms of a family history,
both her mother and brother had osteoarthritis. However, she continues
to live a healthy lifestyle doing much lighter exercise several times a week.
In addition, she has two grown up children, she has never smoked and
drinks about 4 to 8 units of alcohol per week. Examination of her joints
showed obvious signs of Heberden’s and Bouchard’s nodes in several of
her fingers, and bilateral subluxation of the CMC joints. She had tender
and mildly swollen MCPs and wrist joints bilaterally. The left hip had
reduced internal and external rotational movement with tenderness. In
addition, both knees had crepitus on movement and she was found to
have coincidental hypermobile joints affecting her elbows, shoulders,
lower back and ankles with pes planus. An outpatient ultrasound scan
(USS) of her hands and wrists showed presence of small osteophytes
affecting her 2nd and 3rd MCP joints bilaterally with Grey scale 2þand
Doppler 2þaffecting those joints and both wrists, the USS confirmed evi-
dence of active synovitis. X-rays of her hands showed reduced joint
spacein the 2nd and 3rd MCP bilaterally without osteophytes plus degen-
erative changes affecting her DIP and PIP joints. Feet X-ray showed
degenerative changes in 1st MTP joints bilaterally, no chondrocalcinosis
was seen in her hands, feet and knees x-rays. Her initial bloods including
FBC, LFT and glucose were normal, and the inflammatory markers were
normal. She was then given an intramuscular injection of 80mg Kenalog
(triamcinolone acetonide) in clinic. Her rheumatoid factor and anti-CCP
antibody were negative, but subsequent investigation showed her ferritin
was elevated to 963.3 mcg/L (normal range 13-150) with raised iron
42.0umol/L (normal range 5.8-34.5) and raised transferrin saturation
79.2% (normal range 15-45). Ultrasound scan of her liver was normal.
Subsequently, the genetic test revealed that she was homozygous for
C282Ymutationconfirming the diagnosis of hereditary haemochromato-
sis. At follow up review, the patient reported an improvement in her joint
pain, swelling and stiffness of her hands. A repeat USS showed similar
findings, but no Doppler signals were detected showing the absence of
inflammation. Despite the improvement, she continued to report of mild
ongoingintermittentswellingaffecting her MCPs and wrists joints, colchi-
cine was prescribed on a prn basis during those acute episodes. She is
suspected to have pseudogout intermittently, but we were unable to
obtain any synovial fluid to confirm the diagnosis. She has been referred
to the local haematology unit and commenced on fortnightly phlebotomy
treatment for her HH. After the treatment, she has been able to get back to
regulariceskating.
Case description: This casehighlig hts theimportance of hereditary hae-
mochromatosis closely mimicking rheumatoid arthritis. It also shows the
importance of using musculoskeletal USS in routine daily practice with a
high index of suspicion in diagnosing this condition early to prevent future
complications. This patient was known to have osteoarthritis mainly
affecting her hands, knees and left hip, presented with additional joint
swelling affecting her hands and wrists, clinically it was difficult to differ-
entiate it from rheumatoid arthritis. USS showed evidence of active syno-
vitis, but this may have been due to inflammatory osteoarthritis or
pseudogout; however, we did not manage to aspirate any synovial fluid
from her joints to confirm diagnosis. Pseudogout is known to be associ-
atedwithHH, but x-rays of her hands, feet and knees showed no evidence
of chondrocalcinosis. Furthermore, thex-ra yof her hands showed no evi-
dence of the typical hooked osteophytes in her MCP 2 and 3, only mild
reduction in joint space which can occur in rheumatoid arthritis. Overall, it
iseasy to see how in thiscase,the early diagnosis of HH can beoverlooked
and seronegative RA may be made. This patient did not have the typical
presentations such as abnormal FBC, LFT or liver disease, diabetes, skin
pigmentation, heart failure nor a family history. However, early diagnosis
with early treatment will help to prevent these complications in both the
patient and other affected family members. As such, it is imperative we
aimfor earlier diagnosis in cases such as these.
Key learning points: Hereditary haemochromatosis can present with
joint symptoms closely mimicking the features of early rheumatoid arthri-
tis. The key to diagnosis in this case was the use of USS and high index of
suspicion. The key take home message would be that early diagnosis of
hereditary haemochromatosis and early treatment can prevent multiple
complications associated withthiscondition.
23. MULTI-SITE AVASCULAR NECROSIS OF IDIOPATHIC
AETIOLOGY
Dr Tareg Mudawi, Whipps Cross Hospital, London
Dr Arti Mahto, James K Ruffle and Dr Judith Bubbear, Whipps
Cross University Hospital, Barts Health NHS Trust, London
Introduction: Avascular necrosis is defined as the death of bone tissues
secondary to lack of inadequate vascular supply. Its aetiology is
numerous, and sometimes multifactorial, but in essence may be caused
by any perturbation in bony vasculature 1. Such examples range from
fracture, joint dislocation, slipped and epiphysis, to more complex dis-
eases such as sickle cell anaemia, rheumatoid arthritis and systemic
lupus erythematosus. Furthermore, exogenous causes such as alcohol
abuse or corticosteroid administration 1,2 are also apparent, although
with a caveat that in 25% of cases the cause remains unknown. All in all,
thisinturnleadsto bone necrosis, which subsequently may lead to osteo-
chondritis dissecans, should articular joint cartilage become involved.
The most common affected sites are the following 3: 1) head of femur; 2)
neck of talus or 3) scaphoid 1,2. After the bodily insult, anoxia of haemato-
poieticcells causes bonycell death within12-48 hours, with bonemarrow
fatcell death within 5 days.
Case description: Herein we present an interesting case of a 41 year old
male accountant, of origin from Tenerife. This gentleman presented as a
GP referral to our outpatient rheumatology clinic earlier this year with right
sided hip pain and general polyarthralgia. On direct questioning, he was
afflictedwiththis for the prior 3-4 years, affecting his knees, ankles, wrists,
fingers and neck. He furthermore disclosed morning stiffness and diffi-
culty in long distance mobility, but no joint swelling. He was taking over
the counter analgesia only, to minimal avail, and was a never-smoker,
non-drinker. Serologically, his inflammatory markers were negative, and
all rheumatological screen was negative. Screening for haemaglobinop-
athy, myeloma, infection and bone profile were all normal/ within normal
range limits. On examination, these gentleman exhibited limited range-
of-movement (ROM) to his ankles bilaterally, with tenderness. He further-
more displayed tenderness to knee palpation with mild crepitus, albeit to
a full ROM. His right hip was particularly tender, with limited ROM, as was
his left wrist and chest wall. With this gentleman’s clinical findings, he was
subsequently scheduled for X-Ray imaging of his left wrist, ankles (bilat-
erally) and hip. Strikingly, the X-ray imaging revealed avascular necrosis
tothe following: 1) left scaphoid, 2) talus/calcaneum bilaterally and 3) fem-
oral head, also bilaterally. Thus, this gentleman presented with all 3 of the
top three sites for avascular necrosis. For medical management, he was
subsequently given NSAID/further analgesia, zolendronic acid infusion
(to no improvement) and referred to the orthopaedic team for considera-
tionof joint replacement/resu rfacing.
Discussion: Avascular necrosis typically affects one joint in a given indi-
vidual for a presenting complaint. However, in this interesting case we
present ayoung gentleman with 5 affected joints to avascular necrosis, all
of which engendering the ‘top three’ most common sites . It is typically
diagnosed by X-Ray, MRI with or without bone scan, and treatm ents are
wide and encompass firstlymed ical,such as with the use of non-steroidal
anti-inflammatory drugs (NSAIDS), bisphosphonates and secondary
prevention with statins/anticoagulation as required 3,4. Following which,
surgical options are also apparent, and include core decompression,
bone grafting, osteotomy or arthroplasty. Although this patient did not
respond to zolendronic acid infusion, he is now under consideration for
prospective iloprostinfusions5.
Key learning points: Avascular necrosis has a myriad of causes,
although in excess of 25% of cases they remain idiopathic. The most
common sites affected are that of the femur, scaphoid and ankle, with
investigations to confirm this dependant on radiology. Management
comprises that of combined therapy with physiotherapy, medical man-
agement,withconsider ationtosurgicalintervention.
24. COMPLEX REGIONAL PAIN SYNDROME- A
RHEUMATOLOGIST’S DIAGNOSTIC AND MANAGEMENT
CHALLENGE
Dr Geetha Lakshmi Janakiraman, Northern Deanery, Newcastle
upon Tyne
Dr Shikoh Shahzad and Dr Fiona Clarke, James Cook University
Hospital, South Tees Hospitals NHS Trust, Middlesborough
Introduction: Complex regional pain syndrome (CRPS) is a chronic,
debilitating pain with motor, sensory and autonomic symptoms in unilat-
eral limb which may result from previous trauma. Diagnosis is often clini-
cal and treatment is multidisciplinary. Diagnosis and management is
more challenging in paediatric patients presenting with multiple overlap-
pingsymptoms and psychosocialproblems.
Case description: 15 year old girl presented to Paediatrics with 4 month
historyof leg and foot pain and feeling heavy after tripping over a dog while
jogging. She had intense pain resulting in bouts of spasms in her left leg
with skin hypersensitivity which resulted in her using wheelchair and
crutches. This had an immense impact on her mobility and school attend-
ance. Her background included gastritis and Penicillin allergy. She
received 6 weeks of intense physiotherapy and analgesics. She was
referred CAMHS and seen weekly for few months and symptoms
improved with Gabapentin. Up until the age of 17 she remained well with-
out any aids or medications, restarted college and horse-riding. During
i14 11–12 October 2017 POSTER CASE REPORTS
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