Q: Which radiology sign is represented? show answer
Osteopetrosis - autosomal dominant
Sub-optimal growth with chronically ill child with organomegaly. We elicited a clinical history of progressive deafness and blindness.
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BWBA = Bone Within Bone Appearance is A classic radiological sign , and common spotter radiograph / case study for discussion in Postgraduate exams .
Please See Radiopedia / Refer Diffrential Diagnosis Texts for "pathognomic Radiological sign" of 'Bone with in A Bone Appearance'
In our case who was treated outside as for provisional diagnosis of thalassemia / sickle cell anemia in view of organomegaly and chronic illness and anaemia ;
However on hematological investigations the provisional diagnosis was ruled out and based on classical radio-logical sign and further clinical history & insight ( progressive deafness and blindness) the diagnosis of " Autosomal dominant osteopetrosis " was suggested and was confirmed later from treating physician/pediatrician .
Take home message : Importance is of not only recognizing "classic radio-logical sign " but eliciting clinical history with correlation of organomegaly and signs and symptoms yields best diagnosis and aid in proper clinical outcomes i.e. highlights role of Clinical Radiologist