Presentation
Pain in joint, left thumb with history of congenital left hand deformity
Patient Data
Severe hypoplasia of the 3rd, 4th and 5th digits with rudimentary appearing digits, absent ossifications centers of the phalanges, and hypoplastic and dysplastic 3rd, 4th and 5th metacarpals.
Moderate hypoplasia of the 2nd digit with absent proximal and middle phalanx, and severely hypoplastic distal phalanx.
Mildly hypoplastic thumb with wasting of the soft tissues distally.
Mild distal diffuse disuse osteopenia mainly in the digits.
No acute osseous findings. No arthritic changes. No abnormal soft tissue calcifications.
Case Discussion
Amniotic band syndrome (ABS) is a rare congenital disorder which involves fetal entrapment in strands of amniotic tissue. It has a wide spectrum of deformities depending on which part gets entrapped and at what stage of gestation.
In the case presented here the constellation of findings suggest severe congenital deformity of the left hand due to Amniotic band syndrome.
This case was submitted with supervision and input from:
Soni C. Chawla, M.D.
Associate Professor
Department of Radiological Sciences
David Geffen School of Medicine at UCLA
Olive View - UCLA Medical Center