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The Nature of Foot Ray Deficiency in Congenital Fibular Deficiency

Reyes, Bryan A. MD*; Birch, John G. MD, FRS(C); Hootnick, David R. MD; Cherkashin, Alex M. MD; Samchukov, Mikhail L. MD

Journal of Pediatric Orthopaedics: July/August 2017 - Volume 37 - Issue 5 - p 332–337
doi: 10.1097/BPO.0000000000000646
Foot and Ankle
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Background: Absent lateral osseous structures in congenital fibular deficiency, including the distal femur and fibula, have led some authors to refer to the nature of foot ray deficiency as “lateral” as well. Others have suggested that the ray deficiency is in the central portion of the midfoot and forefoot.

We sought to determine whether cuboid preservation and/or cuneiform deficiency in the feet of patients with congenital fibular deficiency implied that the ray deficiency is central rather than lateral in patients with congenital fibular deficiency.

Methods: We identified all patients with a clinical morphologic diagnosis of congenital fibular deficiency at our institution over a 15-year period. We reviewed the records and radiographs of patients who had radiographs of the feet to allow determination of the number of metatarsals, the presence or absence of a cuboid or calcaneocuboid fusion, the number of cuneiforms present (if possible), and any other osseous abnormalities of the foot. We excluded patients with 5-rayed feet, those who had not had radiographs of the feet, or whose radiographs were not adequate to allow accurate assessment of these radiographic features. We defined the characteristic “lateral (fifth) ray present” if there was a well-developed cuboid or calcaneocuboid coalition with which the lateral-most preserved metatarsal articulated.

Results: Twenty-six patients with 28 affected feet met radiographic criteria for inclusion in the study. All affected feet had a well-developed cuboid or calcaneocuboid coalition. The lateral-most ray of 25 patients with 26 affected feet articulated with the cuboid or calcaneocuboid coalition. One patient with bilateral fibular deficiency had bilateral partially deficient cuboids, and the lateral-most metatarsal articulated with the medial remnant of the deformed cuboids. Twenty-one of 28 feet with visible cuneiforms had 2 or 1 cuneiform.

Conclusions: Although the embryology and pathogenesis of congenital fibular deficiency remain unknown, based on the radiographic features of the feet in this study, congenital fibular deficiency should not be viewed as a global “lateral lower-limb deficiency” nor the foot ray deficiency as “lateral.”

Level of Evidence: Level IV—prognostic study.

*University of Texas Southwestern Medical Center

Texas Scottish Rite Hospital for Children, Dallas, TX

State University of New York Upstate Medical University, Syracuse, NY

Acknowledgments of support: None.

J.G.B., A.M.C., and M.L.S. receive royalties from Orthofix for sales of the Truelok circular external fixator. The remaining authors declare no conflicts of interest.

Reprints: John G. Birch, MD, FRS(C), Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, TX 75219. E-mail: john.birch@tsrh.org.

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