Figure 1.
Mucopolysaccharidosis (MPS) Type I-S. A 5-year-old female has bilateral calcaneovalgus deformities of the feet, blue sclera and corneal clouding. She is neurologically normal. She has received bone marrow transplantation (BMT) from a human leukocyte antigen (HLA) identical sibling. Her post-BMT course is uneventful with rapid stable engraftment. (a) Anteroposterior (AP) view of wrist and forearm shows classic features of MPS. Note the short and wide metacarpals with proximal pointing (black arrows). Note the Madelung's deformity of the hands with tilting of distal radii and ulna towards each other (white arrows). (b) AP view of feet shows bilateral calcaneovalgus deformities of the feet. (c) MRI brain T2 weighted axial image shows prominent perivascular spaces with cribriform appearance (black arrows). She is neurologically normal with normal intelligence.