Zeitschrift für Knochenforschung
Offener Zugang

Abstrakt

Hemihypertrophy Spectrum

Heba Elawady and Tamer Ragab

Background: Hemihypertrophy is a condition in which one side or part of the body is larger than the other. The asymmetry can range from mild to severe. It is important to establish a diagnosis because hemihypertrophy is associated with an increased risk for embryonal tumors, mainly Wilms tumor and hepatoblastoma.
Patients and Methods: This study presents ten Egyptian children with variable extent of congenital hemihypertrophy. It included 5 males and 5 females ranging in age from 2 months to 13 years. Abdomino-pelvic ultrasonography, echocardiography, brain MRI and radiological assessment of apparent and possibly hidden bone hypertrophy were performed to all cases.
Results: Cases were classified into Isolated hemihypertrophy (IH) (5 cases), part of overgrowth syndromes (3 cases) and hemihypertrophy with other malformations not fitting any of the known overgrowth syndromes (2 cases). IH cases were subclassified into simple hemihypertrophy (3 cases) and complex hemihypertrophy (2 cases). All cases were sporadic. None of our cases showed malignant transformation.
Conclusion: Hemihypertrophy may be isolated or associated with other congenital malformations. Most isolated cases are sporadic in inheritance with low recurrence risk. Screening for whole body systems is important to detect visceromegaly or other congenital anomalies. Follow up is essential to help in better diagnosis, counseling regarding the course of the disease and the recurrence risk and for early detection of malignancies. Molecular studies will help early diagnosis and distinguishing different hemihypertrophy syndromes.