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December 1993, Vol 147, No. 12 >
Article | December 1993

Radiological Case of the Month FREE

Craig S. Mitchell, DO; Beverly P. Wood, MD
[+] Author Affiliations

Accepted for publication October 30, 1992.

Reprint requests to Department of Radiology, MS81, Childrens Hospital Los Angeles, 4650 Sunset Blvd, Los Angeles, CA 90027 (Dr Wood).


Am J Dis Child. 1993;147(12):1335-1336. doi:10.1001/archpedi.1993.02160360077023.
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A 7-year-old boy was admitted with a 1-month history of diminishing lower extremity strength and progressive loss of bowel and bladder control. He had been hospitalized 2 months earlier with similar symptoms. At that time, a benign paraspinous angiolipomatous tumor producing extradural spinal cord compression was diagnosed and successfully treated with angiographic embolization followed by decompressive laminectomy. Physical examination on admission revealed a frail, thin boy with a large subcutaneous mass involving the left chest wall and extending from the left nipple to the left axilla. Multiple smaller subcutaneous masses were also present on both arms and legs. Areas of increased skin pigmentation were scattered over his body, and a patch of roughened scalelike skin was present on his neck. Fixed deformities of the feet and ankles were present. Lower extremity strength was diminished. A chest roentgenogram (Fig 1), computed tomogram of the chest (Fig 2), magnetic resonance image of

REFERENCES

1 +
Wiedemann HR, Burio GR, Aldenhoff P, Kunze J, Kaufmann HJ, Schirg E.  The Proteus syndrome . Eur J Pediatr . 1983;;140:5-12.
2 +
Viljoen DL, Nelson MM, deJohng G, Beighton P.  Proteus syndrome in southern Africa: natural history and clinical manifestations in 6 individuals . Am J Med Genet . 1987;;27:87-97.
3 +
Costa T, Fitch N, Azouz EM.  Proteus syndrome: report of two cases with pelvic lipomatosis . Pediatrics . 1985;;76:984-989.
4 +
Azouz EM, Costa T, Fitch N.  Radiologic findings in the Proteus syndrome . Pediatr Radiol . 1987;;17:481-485.
5 +
Mucke J, Willgerodt H, Kunzel R, Brock D.  Variability in the Proteus syndrome: report of an affected child with pelvic lipomatosis . Eur J Pediatr . 1985;;143:320-323.
6 +
Clark RD, Donnai D, Rogers J, Cooper J, Baraitser M.  Proteus syndrome: an expanded phenotype . Am J Med Genet . 1987;;27:99-117.
7 +
Tibbles JAR, Cohen MM.  The Proteus syndrome: the Elephant Man diagnosed . BMJ . 1986;;293:683-685.
8 +
Happle R.  Cutaneous manifestations of lethal genes . Hum Genet . 1986;;72:280.

Figures

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Wiedemann HR, Burio GR, Aldenhoff P, Kunze J, Kaufmann HJ, Schirg E.  The Proteus syndrome . Eur J Pediatr . 1983;;140:5-12.
2 +
Viljoen DL, Nelson MM, deJohng G, Beighton P.  Proteus syndrome in southern Africa: natural history and clinical manifestations in 6 individuals . Am J Med Genet . 1987;;27:87-97.
3 +
Costa T, Fitch N, Azouz EM.  Proteus syndrome: report of two cases with pelvic lipomatosis . Pediatrics . 1985;;76:984-989.
4 +
Azouz EM, Costa T, Fitch N.  Radiologic findings in the Proteus syndrome . Pediatr Radiol . 1987;;17:481-485.
5 +
Mucke J, Willgerodt H, Kunzel R, Brock D.  Variability in the Proteus syndrome: report of an affected child with pelvic lipomatosis . Eur J Pediatr . 1985;;143:320-323.
6 +
Clark RD, Donnai D, Rogers J, Cooper J, Baraitser M.  Proteus syndrome: an expanded phenotype . Am J Med Genet . 1987;;27:99-117.
7 +
Tibbles JAR, Cohen MM.  The Proteus syndrome: the Elephant Man diagnosed . BMJ . 1986;;293:683-685.
8 +
Happle R.  Cutaneous manifestations of lethal genes . Hum Genet . 1986;;72:280.

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