Overgrowth of the limbs: A prospective study of 21 Ghanaian patients

Abstract

Introduction: Overgrowth or gigantism is a congenital enlargement of the soft tissue parts of a limb often with associated enlargement of the skeleton, due to overgrowth of one or more cell types. It may be associated with lipofibromatosis, neurofibromatosis, hyperostosis, or hemihypertrophy. Extent of the burden is unknown locally hence this study.
Patients and Methods: A prospective study from January 2005 to December 2009 was undertaken at Komfo Anokye Teaching hospital in Kumasi to document the prevalence and management of patients presenting with congenital enlargement of a limb or digit. The patients were examined clinically and the site of enlargement was recorded. X-rays, pre- and post-operative photographs were taken.
Results: Twenty one patients presented with overgrowth of the limbs during the study period, comprising nine males and 12 females. Age range was from 0.3 to 15 years and mean (S.D.) was 7.6 (4.9) years. Twenty eight sites were affected: 16 involving the upper limb; 12, the lower limb, 16 limbs on the right and 12 on the left. The hand was the most affected site (28.7%); followed by the foot (14.3%). The big toe was the most affected digit (10.7%). Debulking was the commonest procedure (71.4%), followed by ray reduction (17.1%) and ray amputation (11.4%).
Conclusion: Whereas early surgical intervention is recommended, most of the patients presented late and were treated by extensive debulking, ray reduction and ray amputation. 

Keywords: gigantism, lipofibromatosis, neurofibromatosis, debulking