Six year old girl child presented with difficulty in turning neck for last two months.Movement of neck in all directions were painful , and was progressively increasing.
Occasional low grade fever,which was controlled with paracetamol.
No neck swelling or swelling elsewhere noticed by parents.
No history of trauma
No history of bone or joint pain elsewhere .
No skin rashes or bleeds.
No past history of significant illness .
No contact with tuberculosis.
Immunized update
She was pale No lymph node enlargement .
Movement of neck restricted in all directions and torticollis present
She does nt have any
swelling or pain on any othe bony points
Abdomen exam
No hepato splenomegaly
Abdomen exam
No hepato splenomegaly
We did a neurological
examination ............Normal .
IMAGINGS
IMAGINGS
Xray Neck lateral view
First look it may be passed of as normal . But few abnormalities
Common mistakes in interpretation of Xray neck ( both lateral and PA )
1. Missing abnormalities in the lowest cervial vertebra. If not taken properly the shoulder shadow may mask the vertebra
2. Missing features of Axis and atlas and the relation
Before looking at the bodies try to draw a line along anterior posterior margin of bodies. canal and the spine. Usually it ll be possible
Here there is straightening . Inter vertebral space normal , Bodies look normal except the fourth . Axis looks denser . Ring of atlas and atlanto axial relation normal
Seeing this X Ray , she was re examined for bone and joint problems .It showed found out small swelling on forehead and painful areas on her scalp. There was pediculosis but no infected lesions to account for the pain
Plane Xray skull lateral view
Erosion of skull bones multiple sites ,without discrete margins sort of moth eaten appearance..Areas of sclerosis .
MRI brain and Spine
MRI brain and Spine
Look at the Skull bones ..
Brain parenchyma , Pituitary Hypothalamic area was normal
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Cervical spine . the atlas and axis are involved. Lower level multiple vertebral bodies , thoracic and lumbar involved.
No evidence of compression on spinal cord or medulla .
Reexamination CNS was done ,All deep tendon reflexes normal ,sensations normal
Two common entities at
this ageReexamination CNS was done ,All deep tendon reflexes normal ,sensations normal
1. Neuroblatoma
2,Langerhan cell disorder .
Eosinophilic granuloma
3 Chronic recurrent multifocal osteomyelitis
Other Investigations
3 Chronic recurrent multifocal osteomyelitis
Other Investigations
Blood , Hb 6 grams, Total count 8000, N 60 ,L38,E2
Mantauxe negative
Peripheral smear showed microcytic hypochromic RBCS
WBC series and platelet count and distribution were normal
Mantauxe negative
Peripheral smear showed microcytic hypochromic RBCS
WBC series and platelet count and distribution were normal
VMA 24 hour
urine.....was normal
USG abdomen . Liver , spleen normal ,No Mass Adrenals normal .
Xray chest .No rib erosion, No mass in the chest. lung and heart shadow and
mediastinum were normal
Needle aspiration cytology Not done
Needle aspiration cytology Not done
Bone marrow done ...., No evidence of leukemia , lymphoma or neuroblastoma.
Chronic Recurrent Multifocal Osteomyelitis is a rare entity
( CRMO usually involves long bones than skull and spine even though it is possible . Here most of the long bones spared .Examination of clavicle did nt show any swelling or tenderness . Xray showed clavicles were normal .Clavicle is one of the commonest bone involved in CRMO)
We considered Langerhans cell disorders as first possibility
( CRMO usually involves long bones than skull and spine even though it is possible . Here most of the long bones spared .Examination of clavicle did nt show any swelling or tenderness . Xray showed clavicles were normal .Clavicle is one of the commonest bone involved in CRMO)
We considered Langerhans cell disorders as first possibility
.
She was referred to Regional Cancer center and she is on treatment from there
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