Hemimelia
| Hemimelia | |
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| Bones of lower extremity. | |
| ICD-10 | Q73.8 |
| MeSH | D004480 |
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Overview
Fibular Hemimelia or "Longitudinal fibular deficiency" is "the congenital absence of the fibula and it is the most common congenital absence of long bone of the extremities."[1] It is the shortening of the fibula at birth, or the complete lack thereof. The disorder can be noted by ultrasound in utero, allowing for parents to decide on abortion or to prepare for amputation after birth or complex bone lengthening surgery. The amputation usually takes place at 6-months with removal of portions of the legs to retro fit them for prosthetic use. The other treatments which include repeated corrective osteotomies and leg-lengthening surgery are costly and associated with residual deformity.[2]
Common Facts
- It is among most frequent limb anomalies is partial or total absence of fibula;[3]
- it is most common long bone deficiency and is the most common skeletal deformity in the leg;[4]
- most often is unilateral;[5]
- paraxial fibular hemimelia is the most common manifestation (only the postaxial portion of the limb is affected)
- commonly seen as complete terminal deficiency (lateral rays of the foot are affected as well);[6]
- hemimelia can also be intercalary in which case the foot remain unaffected;[7]
- it is prudent to remember that although congenital absence of fibula is evident, this condition is actually a total limb involvement;[8]
- males are affected twice as often as females in most series; [9]
See also
- Bone
- Congenital abnormality
- List of congenital disorders
- ICD-10 Chapter Q: Congenital malformations, deformations and chromosomal abnormalities
- List of ICD-9 codes 740-759: Congenital anomalies
- Mitochondrial disease
- March of Dimes
References
- ↑ Fibular hemimelia: a case report. Nigerian journal of clinical practice 2007;10(3):259-61.
- ↑ Fibular Hemimelia: A New Classification System.
- ↑ http://www.wheelessonline.com/ortho/fibular_hemimelia_longitudinal_fibular_deficiency
- ↑ http://www.wheelessonline.com/ortho/fibular_hemimelia_longitudinal_fibular_deficiency
- ↑ http://www.wheelessonline.com/ortho/fibular_hemimelia_longitudinal_fibular_deficiency
- ↑ http://www.wheelessonline.com/ortho/fibular_hemimelia_longitudinal_fibular_deficiency
- ↑ http://www.wheelessonline.com/ortho/fibular_hemimelia_longitudinal_fibular_deficiency
- ↑ http://www.wheelessonline.com/ortho/fibular_hemimelia_longitudinal_fibular_deficiency
- ↑ http://www.wheelessonline.com/ortho/fibular_hemimelia_longitudinal_fibular_deficiency
Severe tibial growth retardation in total fibular hemimelia after limb lengthening. Journal of Pediatric Orthopaedics, 2003 [1]
External links
- Journal of Joint Bone Sugary 1997 Jan;79(1):58-65. http://www.jbjs.org.uk/cgi/reprint/79-B/6/1040
Template:Phakomatoses and other congenital malformations not elsewhere classified