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Pediatric Radiology

, Volume 40, Issue 3, pp 372–372 | Cite as

Macrodystrophia lipomatosa

  • Jonathan R. DillmanEmail author
  • Peter J. Strouse
Clinical Image

Keywords

Visual Inspection Median Nerve Vascular Lesion Radiographic Finding Neurofibromatosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
A 5-year-old girl presented with left hand enlargement since birth. Visual inspection revealed left index and middle finger macrodactyly. Left hand radiographs demonstrated marked osseous and soft-tissue overgrowth of the second (index) and third (middle) digits (Figs. 1 and 2), consistent with macrodystrophia lipomatosa in a median nerve distribution. Areas of soft-tissue overgrowth appeared radiolucent, suggesting the presence of fat. The right hand was normal.
Fig. 1

Oblique radiograph, left hand

Fig. 2

Lateral radiograph, left hand

Macrodystrophia lipomatosa is a rare nonhereditary form of focal gigantism that is characterized by osseous and fibroadipose tissue overgrowth [1, 2]. This condition affects either the hand or foot, classically occurring in a median or plantar nerve distribution [1, 2]. Distal and volar digital involvement is typical (as in our case), and affected digit growth usually ceases after puberty [1]. Although the radiographic findings of this condition are generally pathognomonic and further evaluation with MRI is usually not necessary, macrodactyly can be due to numerous other causes, including Proteus syndrome, neurofibromatosis, and a variety of vascular lesions [1, 2].

References

  1. 1.
    Goldman AB, Kaye JJ (1977) Macrodystrophia lipomatosa: radiographic diagnosis. AJR 128:101–105PubMedGoogle Scholar
  2. 2.
    Fritz TR, Swischuk LE (2007) Macrodystrophia lipomatosa extending into the upper abdomen. Pediatr Radiol 37:1275–1277CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  1. 1.Department of Radiology, Section of Pediatric RadiologyUniversity of Michigan Health System, C. S. Mott Children’s Hospital, F3503Ann ArborUSA

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