RECENT TRENDS IN MANAGEMENT OF FIBULAR HEMIMELIA
Ahmed Mahmoud Badr Eldeen;
Abstract
Fibular hemimelia is the most common longitudinal deficiency in which the tibia is usually short with an axis deviation (anterior bowing and valgus deformity).(2)
It is not an isolated anomaly but rather a spectrum of dysplasia of the limb. The clinical spectrum includes partial or complete absence of the fibula with variable other anomalies, including tibial shortening and angular deformities, ball and socket ankle, tarsal anomalies, absence of the lateral rays of the foot, femoral shortening, proximal femoral focal deficiency, and occasionally hand anomalies.(5) The major functional deficits are severe shortening of the extremity and equinovalgus deformity of the ankle, knee valgus deformity and flexion contracture, anteroposterior instability of the knee and ankle.(5,6)
It is theorized that a defect in the femoral-fibular-ulnar developmental field can result in a clinical spectrum ranging from complete absence of the limb or proximal femoral focal deficiency to a missing toe and simple tarsal coalition.(11)
Several classifications were proposed to address the deformity, with the Achterman–Kalamchi (12) being the most commonly used.(13)
Fibularhemimeliaclassificationshavebeendescribedbymanyauthors:
• CoventryandJohnson(3)classification(1952).
• FrantzandO‘Rahilly(4)classification(1961).
• AchtermanandKalamchi(12)classification(1979).
• Catagni(33)classification(1991).
• StanitskiandStanitski(34)(2003).
• Paleyclassification(35)(2004).
• Birch (Functional)(37) classification(2011).
Considering the variability of the condition, it is little surprise that over the years many methods have evolved to address the situation.(38)
Despite improvements in surgical techniques and better understanding of the characteristics of this type of congenital deformity, a considerable number of persisting malformations seems to remain after treatment. Parents should have the right expectations and in this instance they will be more co-operative and have better understanding in case of additional required procedures. Fibular hemimelia is very often associated with several concurrent deformities and needs meticulous treatment planning. Better knowledge of the expected problems after initial treatment might be helpful in appropriate treatment planning and in gaining better final results.(95)
Despite a high percentage of complications, tibia elongation with axis and foot correction may be an alternative to amputation.(16)
It is not an isolated anomaly but rather a spectrum of dysplasia of the limb. The clinical spectrum includes partial or complete absence of the fibula with variable other anomalies, including tibial shortening and angular deformities, ball and socket ankle, tarsal anomalies, absence of the lateral rays of the foot, femoral shortening, proximal femoral focal deficiency, and occasionally hand anomalies.(5) The major functional deficits are severe shortening of the extremity and equinovalgus deformity of the ankle, knee valgus deformity and flexion contracture, anteroposterior instability of the knee and ankle.(5,6)
It is theorized that a defect in the femoral-fibular-ulnar developmental field can result in a clinical spectrum ranging from complete absence of the limb or proximal femoral focal deficiency to a missing toe and simple tarsal coalition.(11)
Several classifications were proposed to address the deformity, with the Achterman–Kalamchi (12) being the most commonly used.(13)
Fibularhemimeliaclassificationshavebeendescribedbymanyauthors:
• CoventryandJohnson(3)classification(1952).
• FrantzandO‘Rahilly(4)classification(1961).
• AchtermanandKalamchi(12)classification(1979).
• Catagni(33)classification(1991).
• StanitskiandStanitski(34)(2003).
• Paleyclassification(35)(2004).
• Birch (Functional)(37) classification(2011).
Considering the variability of the condition, it is little surprise that over the years many methods have evolved to address the situation.(38)
Despite improvements in surgical techniques and better understanding of the characteristics of this type of congenital deformity, a considerable number of persisting malformations seems to remain after treatment. Parents should have the right expectations and in this instance they will be more co-operative and have better understanding in case of additional required procedures. Fibular hemimelia is very often associated with several concurrent deformities and needs meticulous treatment planning. Better knowledge of the expected problems after initial treatment might be helpful in appropriate treatment planning and in gaining better final results.(95)
Despite a high percentage of complications, tibia elongation with axis and foot correction may be an alternative to amputation.(16)
Other data
| Title | RECENT TRENDS IN MANAGEMENT OF FIBULAR HEMIMELIA | Other Titles | الطرق المستحدثة في علاج انعدام النصف الشظوى للطرف السفلى | Authors | Ahmed Mahmoud Badr Eldeen | Issue Date | 2016 |
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