EURORAD ESR

Case 13584

GORHAM disease: a paediatric case report

Author(s)
L. Ben Hassine1, W. Douira-Khomsi1, MA. Belhadj1, I. Abbessi2, H. Louati1, I. Ammar1, L. Lahmar1, S. Bouchoucha3, C. Jalel3, I. Bellagha1

(1) Pediatric Imaging Department
Children’s Hospital
(2) Anatomopathology Department
Salah Azaiez Institute
(3) Pediatric Orthopedic Surgery Department
Children’s Hospital.
Tunis. Tunisia
Email:liliarx@yahoo.fr
 
Patient
male, 3 year(s)
 
 
  • Figure 1
    Frontal and lateral X-ray spine views

    Frontal (1A) and lateral (1B) spine views demonstrating scoliosis (white arrows) with paravertebral opacity (black arrow).

     
    Area of Interest: Spine; Imaging Technique: Conventional radiography; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;
     
     
  • Figure 2
    Axial thoraco-abdominal CT view with contrast

    Axial thoraco-abdominal CT view with contrast showing a large non-enhancing soft tissue mass in the posterior mediastinum, presenting lobulated margins (white arrow).

     
    Area of Interest: Abdomen; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Haematologic diseases;
     
     
  • Figure 3
    Sagittal spine CT views with contrast

    Sagittal CT views in soft tissue (3A) and bone windows (3B) showing extensive spine osteolysis responsible for thoracolumbar deformity (black arrows).

     
    Area of Interest: Spine; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Demineralisation-Bone;
     
     
  • Figure 4
    Axial pelvic CT views

    Axial pelvic CT views in bone (4A) and soft tissue windows (4B) showing multiple lytic confluent lesions within the pelvic girdle (black arrow), muscular (black asterisk) and intracanalar extensions (white asterisk).

     
    Area of Interest: Bones; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Demineralisation-Bone;
     
     
  • Figure 5
    Thoraco-lumbar MRI axial views

    Thoraco-lumbar T1 (5A), T2 weighted axial views (5B and 5C), demonstrating a liquid-like signal mass, white asterisk on T1, black asterisk on T2, posterior mediastinum and paravertebral region (black arrows), left...

     
    Area of Interest: Abdomen; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Cysts;
     
     
  • Figure 6
    Sagittal spine MRI views

    Sagittal T2 (6A), T1 before and after contrast injection (6B, 6C) views, showing spine deformity with vertebrae appearing in low signal on T1, heterogenous high signal on T2, visible enhancement after injection (white...

     
    Area of Interest: Spine; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Demineralisation-Bone;
     
     
  • Figure 7
    Coronal MRI view of the left shoulder

    Coronal T2 weighted image demonstrating multifocal bone dissemination with high signal appearance lesions of the scapulae (white arrow) and left humerus (white arrowhead).

     
    Area of Interest: Bones; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Demineralisation-Bone;
     
     
  • Figure 8
    Axial MRI views of the pelvic girdle

    Axial T2 weighted views showing pelvic girdle hyperintense lesions (black asterisk, 8A) with intra-canalar extension (black arrowhead), acetabular involvement (8B, black arrow).

     
    Area of Interest: Bones; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Demineralisation-Bone;
     
     
  • Figure 9
    Histological view

    (HE X 400) Vascular proliferation with anastomosing thin-walled lymphatic vessels between the remaining bony trabeculae.

     
    Area of Interest: Bones; Imaging Technique: Percutaneous; Procedure: Diagnostic procedure; Special Focus: Demineralisation-Bone;
     
     
  • Figure 10
    Immunohistochemistry study

    Immunohistochemical positivity of the endothelial cells for the antibody D2-40.

     
    Area of Interest: Bones; Imaging Technique: Percutaneous; Procedure: Diagnostic procedure; Special Focus: Demineralisation-Bone;
     
     
Frontal (1A) and lateral (1B) spine views demonstrating scoliosis (white arrows) with paravertebral opacity (black arrow).
 
Axial thoraco-abdominal CT view with contrast showing a large non-enhancing soft tissue mass in the posterior mediastinum, presenting lobulated margins (white arrow).
 
Sagittal CT views in soft tissue (3A) and bone windows (3B) showing extensive spine osteolysis responsible for thoracolumbar deformity (black arrows).
 
Axial pelvic CT views in bone (4A) and soft tissue windows (4B) showing multiple lytic confluent lesions within the pelvic girdle (black arrow), muscular (black asterisk) and intracanalar extensions (white asterisk).
 
Thoraco-lumbar T1 (5A), T2 weighted axial views (5B and 5C), demonstrating a liquid-like signal mass, white asterisk on T1, black asterisk on T2, posterior mediastinum and paravertebral region (black arrows), left pleural effusion (white arrow).
 
Sagittal T2 (6A), T1 before and after contrast injection (6B, 6C) views, showing spine deformity with vertebrae appearing in low signal on T1, heterogenous high signal on T2, visible enhancement after injection (white asterisk).
 
Coronal T2 weighted image demonstrating multifocal bone dissemination with high signal appearance lesions of the scapulae (white arrow) and left humerus (white arrowhead).
 
Axial T2 weighted views showing pelvic girdle hyperintense lesions (black asterisk, 8A) with intra-canalar extension (black arrowhead), acetabular involvement (8B, black arrow).
 
(HE X 400) Vascular proliferation with anastomosing thin-walled lymphatic vessels between the remaining bony trabeculae.
 
Immunohistochemical positivity of the endothelial cells for the antibody D2-40.
 
 
 
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