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MULTIFOCAL BONE INFARCTS IN SICKLE CELL THALASSEMIA
Authored By: Keith Fischer and Dhanashree Rajderkar.
Patient: 15 year old
History: 15 year old African American male patient with Sickle cell thalassemia(hemoglobin S/beta thalassemia), presents with bilateral hip pain, worse on the right side.
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Bone Scan 1

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Bone Scan 2

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Bone Scan 3

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Bone Marrow Scintigraphy 1

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Bone Marrow Scintigraphy 2

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Findings:

Whole body Bone Scintigraphy done after injection of TC-99m MDP on 10/12/10 demonstrated- 

Diffusely increased activity in the left humerus relative to the right humerus that has no correlate in the shoulder radiographs. The diffusely increased activity in the left humerus is new since prior bone scintigraphy studies. Differential include bone infarct and infection.

There is nonspecific increased activity in the left femoral head. Advanced changes of avascular necrosis of left femoral head were seen in pelvic radiograph dated 10/10/2010. The increased activity may be seen during reparative phase of avascular necrosis. Decreased activity in the right femoral head region is compatible with known right hip prosthesis seen in the pelvic radiograph.

 

Whole Body Bone Marrow Scintigraphy done  after injection of TC-99m Sulfur Colloid on10/14/10 demostrated-

There is diffusely diseased radiotracer uptake in the bone marrow of left humeral diaphysis. Bone scan done on 10/12/2010 demonstrated diffusely increased activity in the left humerus. The findings from these two scans are consistent with bone marrow infarction in the left humerus. There are additional multiple foci of decreased marrow activity in the diaphysis of the bilateral femurs, tibias, and spine consistent with marrow infarction. There is also decreased marrow activity of the right ischium, and diffuse reduced activity of the right femoral diaphysis which likely represent marrow infarction and clinically correlates to the patient's increasing right hip pain. In addition, splenomegaly and expanded marrow in appendicular skeleton was visualized compatible with patient's diagnosis of sickle cell disease.

 

Plain Radiograph Findings: 

1. Osteonecrosis of the right humeral head with a lytic component extending to the articular surface.

2. Osteonecrosis of the left humeral head without extension to the articular surface.

3. Stable right total hip arthroplasty.

4. Advanced changes of avascular necrosis in the left-side with femoral head collapse, stable.

5.Endplate changes of sickle cell disease.

DDx:

1. Bone Infarcts.

2. Multifocal abscesses.

3.Diffuse Metastatic disease.

4.Generalised Metabolic disorder.

Diagnosis: Bone Infartcs.
General Discussion:

HbS is the most common hemoglobinopathy in the patients of Sickle cell disease. Approx 7% of the patients have HbSA or HbS Thalassemia.  Bone infarcts, osteomyelitis, bone marrow necrosis and aseptic necrosis are common complications of sickle cell disease.Patients with sickle cell anaemia are prone to both infarctive and infective crises. Bone infarction is, however, the hallmark of sickle cell disease.

In patients with sickle cell disease, bone infarcts are typically multiple during the course of a sickle cell crisis. Areas of infarction are demonstrated as photopenic foci within 72 hours after the crisis. Bones commonly involved include metaphysis and subchondrium of long bones, vertebral end-plates, and the heads of femur and humerus.

Bone scan is useful in the acute phase of marrow infartcion.However, patients of SCD have many episodes of infarctions, findings on the bone scan may not be the typical focal areas of decreased uptake due to repeated reparative process, hence are less reliable.Combining bone imaging with marrow scintigraphy is helpful to differentiate a bone infarct from an infection immediately after the occurrence of an infarct. Combination of a large defect on the marrow scan and a smaller defect on the bone scan is typical for an infarct/osteonecrosis.

Diagnostic Criteria: If the bone-marrow scan showed decreased uptake, thought to be indicative of decreased blood flow in the bone marrow, and the bone scan showed abnormal uptake at the site of acute pain, a diagnosis of bone infarction was made. If the bone-marrow scan showed normal uptake and the bone scan showed abnormal uptake, a diagnosis of acute osteomyelitis was made. If neither the bone-marrow scan nor the bone scan showed abnormal uptake, neither diagnosis was made. None of the symptomatic sites had decreased uptake on the bone-marrow scan and normal uptake on the bone scan. However, this combination of findings was noted in asymptomatic locations and was thought to be consistent with the presence of old infarctions.

References:

1. LETTER:  AN UNUSUAL BONE INFARCT IN A YOUNG ADULT SICKLER,  Annals of African Medicine Vol. 4, No. 2; 2005: 91 – 93.

2.The Requisites : Pediatric Radiology 3rd Edition,Pg 176. Johan G. Blickman,Bruce R. Parker,Patrick D. Barnes.

3.eMedicine Specialities>Radiology>Musculoskeletal,Bone Infarct:Imaging, Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, Coauthor(s): Mohammed Jassim Al-Salman, MBBS,  Muthusamy Chandramohan, MBBS, DMRD, FRCR,Ian Turnbull, MB, ChB, MD, DMRD, FRCR, Sumaira MacDonald, MBChB, PhD, MRCP, FRCR; Charles Edward Hutchinson, MD, FRCR.

4. Scientific Article, Differentiation Between Bone Infarction and Acute Osteomyelitis in Children with Sickle-Cell  Disease with Use of Sequential Radionuclide Bone-Marrow and Bone Scans , The Journal of Bone and Joint Surgery (American) 83:1810-1813 (2001),David L. Skaggs, MD, Sam K. Kim, BS, Nathan W. Greene, MD, Deborah Harris, RN and John H. Miller, MD

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Additional Details:

Case Number: 288897Owner(s): Keith Fischer and Dhanashree RajderkarLast Updated: 02-07-2013
Anatomy: Skeletal System   Pathology: Other
Modality: Conventional Radiograph, Nuc MedAccess Level: Readable by all users, writable by NucMed Certifiers
Keywords: bone infarcts

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Certified by Keith Fischer on 06-10-2011

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