Transcript id 1184 ribbing disease

ID 1184
RIBBING DISEASE
• INTRODUCTION:
Ribbing disease is a rare form of sclerosing bone
dysplasia characterised by formation of exuberant
but benign endosteal and periosteal new bone.
It was an uncommon cause of leg pain in post
pubertal young females.
It is either unilateral or asymmetrical and
asynchronously bilateral.
• It affects only long bones especially diaphysis of
tibia and femur.
• Most patients present with pain.
• It is often confused with sclerosing osteomyelitis ,
stress fracture, bone forming tumours or other
sclerosing dysplasia.
• CASE REPORT:
35Yrs old thin built female presented with
complaints of right leg pain for 1 ½ yrs.
There was no history of trauma or fever.
No history of autoimmune disease in her family.
On examination,
There was no clinical signs of infection, no
evidence of bony tenderness.
Hematological parameters and markers of
bone metabolism were normal.
• She underwent radiograph of leg which showed
cortical thickening of diaphysis of right tibia
involving both endosteal and periosteal cortex and
medullary sclerosis. Contralateral tibia and bilateral
femora were normal.
• She further underwent MRI which showed T1,T2
hypointense lesion with bone marrow edema in
right tibial diaphysis.
• Screening CT was done to rule out bone forming
neoplasms. There was no nidus , cortical breach or
fracture line seen in this patient.
Anteroposterior and lateral radiographs of right leg shows cortical
thickening of right tibial diaphysis involving both periosteal and
endosteal cortex and reduction of medullary cavity. Left tibia,
bilateral femora were normal.
MRI –T1 CORONAL
MRI- T1 Coronal image shows cortical thickening of right tibial
diaphysis with hypointense signal in mid diaphysis. Left tibia is normal.
MRI -T2 FAT SAT CORONAL
MRI -T2 FAT SAT Coronal image shows hypointense diaphyseal lesion
with surrounding oedema.
MRI-T2 AXIAL
MRI T2 Axial images show cortical thickening with near complete
occlusion of medullary cavity and bone marrow edema.
CT SCREENING
Coronal CT of leg shows geographic diaphyseal sclerotic areas with
thickened cortex and medullary sclerosis. Left tibia is normal.
CT SCREENING
CT images of right leg show diaphyseal cortical thickening (homogenous
increased density)involving both endosteal and periosteal cortex with near
complete obliteration of medullary cavity. Left tibia is normal.
• DISCUSSION:
In 1947,
RIBBING described a family with 4
out of 6 siblings with diaphyseal sclerosis of long
bones. He designated the term heriditary multiple
diaphyseal sclerosis.
It is an autosomal recessive disease with uncommon
cause of leg pain.
•
Ribbing disease presents with leg pain and
studies demonstrated that marrow edema is
associated and responsible for pain.
• In ribbing disease markers of bone formation and
resorption are normal.
But Tc 99m-MDP scan shows intense irregular uptake
in diaphyseal region of tibia in delayed and blood
pooling phases.
• The lack of elevation of bone turnover markers is
explained by limited amount of skeleton involved.
Area of affected bone is too small to affect overall
bone turnover markers.
• There are many sclerotic bone disorders that can
be confused with Ribbing Disease.
• In the patient who presents with unilateral increased
density and thickness of the tibia, differential
diagnosis includes stress fracture, intramedullary
ostesclerosis , chronic sclerosing osteomyelitis,
osteoid osteoma and bone forming tumours .
• Chronic sclerosing osteomyelitis mainly affects
metaphyseal region of long bones causing bony
sclerosis and expansion.
• Stress fractures have history of overuse and
complaints of pain and swelling over the affected
region. It mainly affects bones of lower leg and foot
and it have visible fracture line.
• Osteoid osteoma have radiolucent nidus.
• Bone forming tumours have bone expansion, soft
tissue components and periosteal reaction.
• Bilateral increased density and thickness of the tibia may
be associated with bone dysplasias such as CamuratiEngelmann disease, Erdhiem Chester disease,
intramedullary osteosclerosis.
• Engelmann-camurati disease is an autosomal dominant
disorder presents during childhood causing bilateral and
symmetrical involvement of diaphysis of long bones and
also affects skull.
• Chester disease has bilateral, symmetrical involvement
and widespread systemic features.
• Intramedullary osteosclerosis has similar imaging features
as ribbing disease but not associated with marrow
edema.
• The treatment of ribbing disease is mainly
symptomatic and done with Non Steroidal AntiInflammatory Drugs. Bisphosphonates have limited
role.
• Medullary rimming is a surgical treatment when
medical management fails.
• CONCLUSION :
Ribbing disease is a rare diaphyseal sclerosing
dysplasia.
With lack of clinical signs of infection and
biochemical evidence of metabolic bone disease,
positive clinical and imaging features helped us to
arrive a correct diagnosis.
References :
• Resnick-Diagnosis of bone and joint disorders 3rd
edition: Volume 6 ,Section XV111 Chapter 93 : 44164420.
• Nishikant AD et al., Ribbing disease: uncommon
cause of a common symptom. Indian J Nucl Med.
Jan-Mar 2011; 26(1):36-39.
• Ozturkmen Y et al., Ribbing disease: a case report
and literature review. Acta Orthop Traumatol Ture.
2011; 45(1): 58-65.
• Seeger LL et al., Ribbing disease(a diaphyseal
sclerosis): Imaging and differential diagnosis. AJR:
september 1996; 167.
• Gaeta M et al., MRI in Ribbing disease- a case
report. ACTA orthopaedica 2009; 80(5):893-99.
• Greenspan A et al.,orthopaedic imaging-A
practical approach.5th edition,page 958, 2011.