Hemihyperplasia syndromes.
OBJECTIVE
Hemihyperplasia is a heterogenous group of disorders characterized by asymmetric limb growth. There is considerable confusion regarding their classification and ascertainment into various syndromes. We tried to look into the various aspects of hemihyperplasia syndromes.


METHODS
Records of 17 consecutive cases of hemihyperplasia were reviewed and were ascertained into various syndromes based on available literature and diagnostic criteria.


RESULTS
Of the 17 cases with hemihyperplasia, 3 cases satisfied the diagnostic criteria for Proteus syndrome. One patient each was ascertained as Klippel Trenaunay Weber syndrome and Hemihyperplasia- Multiple lipomatosis. 9 cases were classified as isolated hemihyperplasia. We found two novel associations with hemihyperplasia; namely Ehlers-Danlos syndrome like skin changes and Poland anomaly on the affected side. The remaining 3 cases had miscellaneous disorders with limb asymmetry, namely Neurofibromatosis Type I in 2 cases and Olliers disease in one case.


CONCLUSIONS
Efforts to diagnose syndromes of hemihyperplasia help in genetic counseling.
Dalal
Ashwin B
AB
Department of Medical Genetics, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh 226014, India.
Phadke
Shubha R
SR
Pradhan
Mandakini
M
Sharda
Sheetal
S
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005121:Extremities / Q000254:growth & development*; D005260:Female; D006130:Growth Disorders / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008297:Male; D013577:Syndrome