A Successful Reconstructive Case of Tibia Hemimelia in Syndromic Child
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2018, Vol 19, Issue 4
Abstract
Tibia hemimelia is a very rare congenital disorder. It is characterized by either absent or deficient of the tibia in relatively normal fibula. Therefore clinically; the affected leg will appear short and the foot will be in equino-varus position. It is infrequently associated with other malformations. We report a case of a syndromic child with unilateral tibia hemimelia, whom underwent series of reconstructive procedures and succeed obtaining good functional outcome. Congenital long bone deficiency is very rare, so as tibia hemimelia, which is reported to be one in one million [1]. The first reported case of tibia hemimelia is in 1861. Tibia hemimelia is usually appears as a short and deformed leg with knee and ankle involvement. The knee involvement may present as absent of extensor mechanism, multidirectional ligamentous laxity or flexion contracture. The ankle typically appears varus and equinous, whereas the foot will be in adduction and supination. There are reported associated syndromes and anomalies such as Werner syndrome, tibia hemimelia diplopodia syndrome, and tibia hemimelia split hand and foot syndrome. Some cases of tibia hemimelia are genetically transmitted as autosomal dominant or recessive, whereas others are sporadic. Jones classification is simple widely used radiographic classification, and further modified by Kalamchi and Dawe in 1985 (Table 1). Recent classification by Paley in 2003 is also based on Jones classification [2]. These classifications play a role to determine an appropriate intervention in terms of limb salvage procedure or amputation. We described a case of VACTERL association (vertebral defects, anal atresia, cardiac defects, trachea-esophageal fistula, renal anomalies, and limb abnormalities) with unilateral tibia hemimelia that successfully reconstructed with good functional outcome.
Authors and Affiliations
Norzakiah Abu Mansor Matardiah, Nor Ashikin Johari, Mohd Azli Abdul Hamid
Keywords
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- EP ID EP622591
- DOI 10.26717/BJSTR.2019.19.003352
- Views 136
- Downloads 0
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