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Familial Onycho-Osteodysplasia FREE

MARK M. RUBENSTEIN, MD; GUNNAR B. STICKLER, MD
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Received for publication Jan 30, 1964.


Am J Dis Child. 1964;107(6):640-643. doi:10.1001/archpedi.1964.02080060642016.
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Introduction  Iliac horns (Fong's lesion) are bilateral, but not always symmetrical, bony iliac protrusions arising from the posterior surfaces of the iliac bones. They are usually about 2.5 cm in diameter, and project about 3 cm laterally and posteriorly; in general, they are easily palpable. Although this abnormality is not rare, with cases being described predominantly in the radiologic literature, reports of this lesion are virtually absent from the pediatric literature. It therefore seemed desirable to bring this hereditary abnormality and its associated malformations involving fingernails and the skeletal system to the attention of pediatricians by reporting a case and briefly reviewing the pertinent literature.

Report of Case  A boy, 810/12 years of age, was brought to the Mayo Clinic in September, 1963, because of recurrent left abdominal pain. He is of Swedish-German descent and his pedigree on the paternal side is presented in Fig 1. At birth he had

REFERENCES

Fong, E. E.:  "Iliac Horns" (Symmetrical Bilateral Central Posterior Iliac Processes) , Radiology 47:517-518 ( (Nov) ) 1946;.
Zimmerman, C.:  Iliac Horns: Pathognomonic Roentgen Sign of Familial Onycho-Osteodysplasia , Amer J Roentgen 86:478-483 ( (Sept) ) 1961;.
Mino, R. A.; Mino, V. H.; and Livingstone, R. G.:  Osseous Dysplasia and Dystrophy of Nails , Amer J Roentgen 60:633-641 ( (Nov) ) 1948;.
Caffey, J.: Pediatric X-Ray Diagnosis , 4th ed, Chicago: Year Book Publishers, Inc., 1961;, pp 1130-1131.
Cottereill, C. P., and Jacobs, P.:  Hereditary Arthro-Osteo-Onychodysplasia Associated With Iliac Horns , Brit J Clin Pract 15:933-941 ( (Nov) ) 1961;.
Thompson, E. A.; Walker, E. T.; and Weens, H. S.:  Iliac Horns (Osseous Manifestation of Hereditary Arthrodysplasia Associated With Dystrophy of Fingernails) , Radiology 53:88-92 ( (July) ) 1949;.
Lawler, S. D.; Renwick, J. H.; and Wildervanck, L. S.:  Further Families Showing Linkage Between ABO and Nail-Patella Loci, With No Evidence of Heterogeneity , Ann Hum Genet 21:410-415, 1956;.
Hawkins, C. F., and Smith, O. E.:  Renal Dysplasia in Family With Multiple Hereditary Abnormalities Including Iliac Horns , Lancet 1:803-808 ( (April 29) ) 1950;.

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References

Fong, E. E.:  "Iliac Horns" (Symmetrical Bilateral Central Posterior Iliac Processes) , Radiology 47:517-518 ( (Nov) ) 1946;.
Zimmerman, C.:  Iliac Horns: Pathognomonic Roentgen Sign of Familial Onycho-Osteodysplasia , Amer J Roentgen 86:478-483 ( (Sept) ) 1961;.
Mino, R. A.; Mino, V. H.; and Livingstone, R. G.:  Osseous Dysplasia and Dystrophy of Nails , Amer J Roentgen 60:633-641 ( (Nov) ) 1948;.
Caffey, J.: Pediatric X-Ray Diagnosis , 4th ed, Chicago: Year Book Publishers, Inc., 1961;, pp 1130-1131.
Cottereill, C. P., and Jacobs, P.:  Hereditary Arthro-Osteo-Onychodysplasia Associated With Iliac Horns , Brit J Clin Pract 15:933-941 ( (Nov) ) 1961;.
Thompson, E. A.; Walker, E. T.; and Weens, H. S.:  Iliac Horns (Osseous Manifestation of Hereditary Arthrodysplasia Associated With Dystrophy of Fingernails) , Radiology 53:88-92 ( (July) ) 1949;.
Lawler, S. D.; Renwick, J. H.; and Wildervanck, L. S.:  Further Families Showing Linkage Between ABO and Nail-Patella Loci, With No Evidence of Heterogeneity , Ann Hum Genet 21:410-415, 1956;.
Hawkins, C. F., and Smith, O. E.:  Renal Dysplasia in Family With Multiple Hereditary Abnormalities Including Iliac Horns , Lancet 1:803-808 ( (April 29) ) 1950;.

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