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Int Orthop. 2008 December; 32(6): 845–846.
Published online 2008 July 9. doi:  10.1007/s00264-008-0601-4
PMCID: PMC2898949

Reply to David R. Hootnick

We thank Dr. Hootnick for his comments and his interest in our paper.

The purpose of our paper was to demonstrate the disease entity which had absent lateral rays and a normal fibula as the mildest form of postaxial hypoplasia of the lower extremity.

Dr. Hootnick thought that the absent rays are not lateral ones but midline ones and that absent rays are not a clinical feature of postaxial hypoplasia but result from vascular agenesis. He mentioned that Fig. 1a, an AP radiograph in our paper, demonstrates on gross inspection absence of the second and third metatarsals. However, we considered the fourth and fifth metatarsals to be absent in this figure for several reasons. First, the fifth metatarsal base on the normal side, which is an insertion site for the peroneus brevis muscle, is different from the most lateral metatarsal base on the affected side. Second, the lateral two toes of the affected side are not curved in the same manner as the fourth and fifth toes of the normal side. Third, if the midline metatarsal is missing, the first metatarsal and lateral one should be divergent because of absence of the transverse metatarsal ligament. Actually most papers dealing with fibular hemimelia indicated that the absent rays are lateral ones [13, 57].

Fig. 1
Anteroposterior radiograph demonstrating absence of lateral rays

Dr. Hootnick [4] demonstrated that there was an association of malformation of bone and arteries in skeletal dysplasia of the lower limb with an absent fibula. It may be that abnormal vascularisation during embryogenesis results in skeletal dysplasia; however, we think more proof is needed for this hypothesis [4]. The hypothesis of abnormal vascularisation may not explain why just the lateral condyle of the distal femur is involved and the fibula is more commonly involved than the tibia, etc. Actually vascular dysgenesis could be one manifestation of the whole musculoskeletal dysplasia of postaxial hypoplasia.

Footnotes

This reply refers to the letter that can be found at http://dx.doi.org/10.1007/s00264-008-0597-9.

References

1. Achterman C, Kalamchi A. Congenital deficiency of the fibula. J Bone Joint Surg Br. 1979;61-B(2):133–137. [PubMed]
2. Farmer AW, Laurin CA. Congenital absence of the fibula. J Bone Joint Surg Am. 1960;42-A:1–12. [PubMed]
3. Frantz CH, O’Rahilly R. Congenital skeletal limb deficiencies. J Bone Joint Surg Am. 1961;43-A:1202–1224.
4. Hootnick DR, Levinson EM, Randall PA, Packard DS., Jr Vascular dysgenesis associated with skeletal dysplasia of the lower limb. J Bone Joint Surg Am. 1980;62:1123–1129. [PubMed]
5. Searle CP, Hildebrand RK, Lester EL, Caskey PM. Findings of fibular hemimelia syndrome with radiographically normal fibulae. J Pediatr Orthop B. 2004;13(3):184–188. doi: 10.1097/00009957-200405000-00008. [PubMed] [Cross Ref]
6. Stevens PM, Arms D. Postaxial hypoplasia of the lower extremity. J Pediatr Orthop. 2000;20(2):166–172. doi: 10.1097/00004694-200003000-00007. [PubMed] [Cross Ref]
7. Wood WL, Zlotsky N, Westin GW. Congenital absence of the fibula. Treatment by Syme amputation; indications and technique. J Bone Joint Surg Am. 1965;47:1159–1169. [PubMed]

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