Ddx for diffuse osteosclerosis (Mnemonic “ROMPS”)
“ROMPS”
Renal osteodystrophy
Osteopetrosis
Metastases, Myelofibrosis
Pyknodysostosis
Sickle cell anemia
Features of Renal Osteodystrophy
- Occurs in children or adults
- “Rugger jersey” spine (ill-defined bands of endplate sclerosis)
- Subperiosteal bone resorption
- Lateral clavicle osteolysis
- Secondary signs of renal failure:
-haemodialysis or peritoneal dialysis lines
-vascular fistulae
-vascular and soft tissue calcifications (transplant kidney shadow)
Features of Osteopetrosis
Paediatric onset
Symmetric, generalised increase in bone density with loss of distinction between the cortical and medullary bone.
“Sandwich” vertebrae (well-defined endplate sclerosis)
Fractures
Sclerotic mandible with supernumerary teeth
Erlenmeyer flask deformity
“Bone in bone” appearance in long bones and spine
Differentiate from Osteopetrosis using a lateral skull and mandible XR: A normal size sclerotic mandible with supernumerary teeth is
present in osteopetrosis, while a hypoplastic sclerotic mandible associated with
multiple wormian bones is present in pyknodysostosis.
Features of Diffuse Metastasis
focal cortical destruction/lytic lesions
lung nodules
lymphangitis carcinomatosis
evidence of surgery, e.g. mastectomy, axillary clips
Common Primary Cancers causing sclerotic mets:
-prostate carcinoma (most common)
-breast carcinoma (may be mixed)
-transitional cell carcinoma (TCC)
-carcinoid
-medulloblastoma
-neuroblastoma
-mucinous adenocarcinoma of the gastrointestinal tract (e.g. colon carcinoma, gastric carcinoma)
-lymphoma (e.g. ivory vertebra)
-small cell lung cancer
-pulmonary adenocarcinoma
-medullary thyroid carcinoma
Features of Myelofibrosis
onset typically in those aged >50 years
narrowed medullary cavity
hepatosplenomegaly/splenectomy (surgical clips)
Features of Pyknodysostosis
Paediatric onset
New Fractures/bony deformity from old #
Hypoplastic sclerotic mandible
Multiple wormian bones
“Pencil-sharpened” distal phalanges
Differentiate from Osteopetrosis using a lateral skull and mandible XR: A normal size sclerotic mandible with supernumerary teeth is
present in osteopetrosis, while a hypoplastic sclerotic mandible associated with
multiple wormian bones is present in pyknodysostosis.
MSK features of sickle cell anaemia
“Codfish”/H-shaped vertebrae (central endplate depressions in multiple thoracic vertebral
bodies, which have a biconcave shape)
Avascular necrosis (AVN) humeral/femoral heads
Skull: widening of diploic space, thinning of the outer table, hair-on-end appearance
Diffuse osteosclerosis
Cranial abnormalities associated with osteopetrosis
Hydrocephalus, optic nerve atrophy,
facial paralysis, deafness, subarachnoid
hemorrhage, and obliteration of the sinuses.
Super dense skull as diploic spaces are obliterated.
Abdominal features of sickle cell anaemia
SPLEEN:
-Transient splenomegaly -> Absent/atrophic calcified spleen by age 5
-Splenic abscesses
HPB:
-hepatic iron deposition secondary to multiple transfusions -> hepatomegaly +/- portal hypertension
Abdominal features of sickle cell anaemia
SPLEEN:
-Transient splenomegaly -> Absent/atrophic calcified spleen by age 5
-Splenic abscesses
HPB:
-hepatic iron deposition secondary to multiple transfusions -> hepatomegaly +/- portal hypertension
- cholelithiasis +/- choledocholithiasis
-multiple liver abscesses
GU:
- kidneys large at first -> will shrink as esrf sets in
- renal papillary necrosis
- renal vein thrombosis
GI:
- approximately 40% of patients may develop peptic ulcers due to reduced mucosal resistance and bowel ischaemia
- girdle syndrome: acute circumferential abdominal pain of vaso-occlusive aetiology
Cerebral manifestations of sickle cell anaemia
- Sickle cell disease is among the most common causes of stroke in the paediatric population.
- tortuosity of intra and extracranial arteries
- intracranial ICA stenosis and occlusion results in moyamoya syndrome
- cerebral atrophy, most common 2
- cerebral ischaemia:
- chronic small vessel ischaemia
- cortical infarcts
- cerebral fat emboli from bone marrow necrosis (starfield pattern) - predilection for aneurysms and subsequent intraparenchymal or subarachnoid haemorrhage
- PRES
