Abstract
Dorsal dimelia (the appearance of dorsal hand structures on the palmar aspect of the hand) and ventral dimelia (the appearance of ventral hand structures on the dorsal aspect of the hand) are rare congenital anomalies of the hand. None of the previously reported cases had combined dorsal and ventral dimelia in the same patient. Here, we report a case of severe ulnar ray deficiency. The hand had two digits: the radial digit had a palmar nail (dorsal dimelia) and the ulnar digit had absence of the normal dorsal nail along with the appearance of an ectopic pulp on the dorsal aspect of the digit (ventral dimelia). Ulnar ray deficiency is an error of sonic hedgehog (SHH) responsible for antero-posterior patterning of the limb in-utero. Ventral and dorsal dimelia are errors of dorso-ventral patterning of the hand. The complex interactions of SHH with the dorso-ventral axis of development may explain the concurrent dimelia in our patient.
Highlights
The appearance of dorsal hand structures on the palmar aspect of the hand is rare and is known as dorsal dimelia
We report a patient with severe ulnar ray deficiency who presented with combined dorsal and ventral dimelia in the same hand
The case we reported is unique because of the appearance of both dorsal dimelia and ventral dimelia in the same patient
Summary
Dorsal dimelia (the appearance of dorsal hand structures on the palmar aspect of the hand) and ventral dimelia (the appearance of ventral hand structures on the dorsal aspect of the hand) are rare congenital anomalies of the hand. None of the previously reported cases had combined dorsal and ventral dimelia in the same patient. We report a case of severe ulnar ray deficiency. The hand had two digits: the radial digit had a palmar nail (dorsal dimelia) and the ulnar digit had absence of the normal dorsal nail along with the appearance of an ectopic pulp on the dorsal aspect of the digit (ventral dimelia). Ulnar ray deficiency is an error of sonic hedgehog (SHH) responsible for antero-posterior patterning of the limb in-utero. Ventral and dorsal dimelia are errors of dorso-ventral patterning of the hand. The complex interactions of SHH with the dorso-ventral axis of development may explain the concurrent dimelia in our patient. Dorsal and Ventral Dimelia in the Same Hand in A Patient with Severe Ulnar Ray Deficiency: A Case Report.
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