Bilateral hand polydactyly with right preaxial syndactyly

Case contributed by Muhammad Ejaz Anjum

Presentation

Bilateral congenital hand deformities. Right thumb intrinsic muscles present, perfusion intact, pinch and grip saw present. Left thumb intrinsic muscles present and normal pinch and grip. Severe limitation of hand function, unable to hold objects and middle finger coming in the way

Patient Data

Age: 2 years
Gender: Male

Left Hand: 

Six separate digits are identified on the left side(numbered from the thumb, lateral to medial).

The left thumb shows normal bones and alignment.

The left second, third, fourth and fifth digits demonstrate normal sized proximal phalanges, short, stubby middle phalanges and variable degree of hypoplasia of the distal phalanges. The 6th digit on the left side shows normal proximal phalanx, there is a hypoplasia of the middle phalanx and absence of ossified bone at the distal phalanx.

There are six metacarpal bones. They are separate and show well-defined contour and morphology. The sixth metacarpal bone is not proportionate in size to the rest of the metacarpals. 

Right Hand:

Seven digits are identified on the right side with the thumb and index finger showing syndactyly. The metacarpal bones of the thumb and index finger are not fused. 

The right third, fourth, fifth, and sixth digits show normal sizes of the proximal phalanges, short stubby middle phalanges and there is variable hypoplasia of the distal phalanges.  The 7th digit on the right side shows normal proximal phalanx.  There is a hypoplasia of the middle phalanx and absence of ossified bone at the distal phalanx

There are variable fusion and pseudo articulation involving the second and third metacarpal bone. The overall morphology of the bone is bizarre. The rest of the fifth and sixth metacarpal bones are separate and show well-defined contour and morphology. The seventh metacarpal bone is not proportionate in size to the rest of the metacarpals. 

On both sides:

The large bizarre shaped carpal bone which cannot be differentiated into a specific type of carpal bone noted.

Three separate ossified carpal bones are also identified in the wrist.

The visualized distal radius and ulna show mild metaphyseal broadening and flattening of the surfaces

 

Case Discussion

This case demonstrates the clear features of bilateral hand polydactyly with right preaxial syndactyly. Clearly visible on AP and Oblique views of hand x-ray.

 

Appreciation is extended to Dr Ajay Prashanth DsouzaJukha Shater Ali Ali AL Badawi, and fellow Radiographers / Radiology Nurses, Department of Radiology, Al Jalila Children's Specialty Hospital, Dubai, UAE, for contributing to this case.

 

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