EURORAD ESR

Case 12659

A rare case of polycarpyly in a patient with Ellis-van Creveld syndrome: plain film findings and additional value of MRI

Author(s)
M. Mespreuve 1, 2, F. Vanhoenacker 1, 2, F. Malfait 3, D. Mortier 4, G. Mertens 5

(1) Department of Radiology, St.-Maarten General Hospital, Leopoldstraat 2, 2800 Mechelen, Belgium
(2) Department of Radiology, University Hospital Ghent, Ghent, Belgium,
(3) Center for Medical Genetics, University Hospital Ghent, Ghent, Belgium,
(4) Department of Orthopedic Surgery, St.-Maarten General Hospital, Mechelen, Belgium,
(5) Department of Pediatrics, St.-Maarten General Hospital, Mechelen, Belgium.

Email:marc.mespreuve@skynet.be
 
Patient
male, 11 year(s)
 
 
  • Figure 1
    Plain radiography of the right hand

    Postaxial polydactyly. V-VI-synmetacarpalism. Hypertrophic proximal and absent middle phalanx. Shortened and broader (white arrows) metacarpals. Dumbbell shaped phalanges (horizontal black arrows). Phalangeal...

     
    Area of Interest: Bones; Imaging Technique: Digital radiography; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 2
    Plain radiography with detail of the right carpus

    Polycarpyly: small Capitatum (C), Hamato-Capitate Fusion Bone (HC) and Fifth Distal Carpal Bone (FDCB).

     
    Area of Interest: Bones; Imaging Technique: Digital radiography; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 3
    Plain radiography of the carpal bones of the right hand

    Hamulus (white arrow). Rounded head articulating with the lunate (small white arrow). The capitate: articulation with the 3rd metacarpal (small vertical black arrow), with a lunate facet (large...

     
    Area of Interest: Bones; Imaging Technique: Digital radiography; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 4
    Plain radiography of the left hand.

    Symmetrical findings, compared to the right side.

     
    Area of Interest: Bones; Imaging Technique: Digital radiography; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 5
    MRI of the right hand. Coronal SE T1-WI

    The capitate bone (C), the hamato-capitate fusion bone (HC) and the fifth distal carpal bone (FDCB) are indicated.

     
    Area of Interest: Bones; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 6
    MRI Axial SE-T1-WI (at the white line in fig. 4)

    The capitate bone (C), the hamato-capitate fusion bone (HC) an the fifth distal carpal bone (FDCB) are indicated.

     
    Area of Interest: Bones; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 7
    MRI of the right hand. Coronal SE T1-WI.

    Note the hamulus on the large fusion bone (white arrow) located at the ulnar side of all the long flexor tendons.

     
    Area of Interest: Bones; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 8
    Coronal SE PD -WI of the right wrist

    The palmar radioscaphocapitate ligament (white arrows) helps to identify the capitate bone. No bone marrow oedema due to malfunctioning, despite the abnormal configuration of the distal carpal row.

     
    Area of Interest: Bones; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 9
    Axial SE T1-WI of the right hand

    The attachment of the thumb adductor muscle (white arrows) is running - as normally - to the 3rd metacarpal base and capitate bone and also to the 4th metacarpal base and HC (argument in favour).

     
    Area of Interest: Bones; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 10
    Overview of clinical features of EvC

    Cardinal features and additional features of EvC.

     
    Area of Interest: Bones; Imaging Technique: Digital radiography; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
Postaxial polydactyly. V-VI-synmetacarpalism. Hypertrophic proximal and absent middle phalanx. Shortened and broader (white arrows) metacarpals. Dumbbell shaped phalanges (horizontal black arrows). Phalangeal acromesomelia (vertical black arrows). Hypoplastic terminal phalanges (oval).
 
Polycarpyly: small Capitatum (C), Hamato-Capitate Fusion Bone (HC) and Fifth Distal Carpal Bone (FDCB).
 
Hamulus (white arrow). Rounded head articulating with the lunate (small white arrow). The capitate: articulation with the 3rd metacarpal (small vertical black arrow), with a lunate facet (large black arrow). Neck groove (horizontal black arrow).
 
Symmetrical findings, compared to the right side.
 
The capitate bone (C), the hamato-capitate fusion bone (HC) and the fifth distal carpal bone (FDCB) are indicated.
 
The capitate bone (C), the hamato-capitate fusion bone (HC) an the fifth distal carpal bone (FDCB) are indicated.
 
Note the hamulus on the large fusion bone (white arrow) located at the ulnar side of all the long flexor tendons.
 
The palmar radioscaphocapitate ligament (white arrows) helps to identify the capitate bone. No bone marrow oedema due to malfunctioning, despite the abnormal configuration of the distal carpal row.
 
The attachment of the thumb adductor muscle (white arrows) is running - as normally - to the 3rd metacarpal base and capitate bone and also to the 4th metacarpal base and HC (argument in favour).
 
Cardinal features and additional features of EvC.
 
 
 
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