Efficacy of MRI in classifying proximal focal femoral deficiency

Maldjian, Catherine; Patel, Tanvi; Klein, Robert M.; Smith, R. V.

doi:10.1007/s00256-006-0218-x

Cited by 32 publications

(18 citation statements)

References 7 publications

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“…It ranges from mild shortening to severe deficiency of the femoral head, acetabulum, and femoral shaft. PFFD diagnosis requires a shortened femur with proximal femoral deficiency and therefore deficiency of the iliofemoral articulation, leg length discrepancy, limb malrotation, and varus deformity at the subtrochanteric level (24). Fifteen percent of cases have bilateral involvement (11).…”

Section: Proximal Focal Femoral Deficiencymentioning

confidence: 99%

Common Patterns of Congenital Lower Extremity Shortening: Diagnosis, Classification, and Follow-up

Bedoya¹,

Chauvin²,

Jaramillo

et al. 2015

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Congenital lower limb shortening is a group of relatively rare, heterogeneous disorders. Proximal focal femoral deficiency (PFFD) and fibular hemimelia (FH) are the most common pathologic entities in this disease spectrum. PFFD is characterized by variable degrees of shortening or absence of the femoral head, with associated dysplasia of the acetabulum and femoral shaft. FH ranges from mild hypoplasia to complete absence of the fibula with variable shortening of the tibia. The development of the lower limb requires complex and precise gene interactions. Although the etiologies of PFFD and FH remain unknown, there is a strong association between the two disorders. Associated congenital defects in the lower extremity are found in more than 50% of patients with PFFD, ipsilateral FH being the most common. FH also has a strong association with shortening and bowing of the tibia and with foot deformities such as absence of the lateral rays of the foot. Early diagnosis and radiologic classification of these abnormalities are imperative for appropriate management and surgical planning. Plain radiography remains the main diagnostic imaging modality for both PFFD and FH, and appropriate description of the osseous abnormalities seen on radiographs allows accurate classification, prognostic evaluation, and surgical planning. Minor malformations may commonly be misdiagnosed.

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Section: Proximal Focal Femoral Deficiencymentioning

confidence: 99%

Common Patterns of Congenital Lower Extremity Shortening: Diagnosis, Classification, and Follow-up

Bedoya¹,

Chauvin²,

Jaramillo

et al. 2015

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“…The growth ratio of the abnormal to the normal limb throughout childhood is usually constant [4]. The Amstutz classification [5], another common classification system, has also been used in PFFD and has been applied to MRI [6].…”

Section: Discussionmentioning

confidence: 99%

“…We concluded that these features of Case 2 represented Aitken class A deformity, the mildest form of PFFD. Maldjian et al [6] used MRI in diagnosis and classification of PFFD, stating that MRI was more accurate than radiographic evaluation for the classification of PFFD, particularly prior to the ossification of cartilaginous components in the femurs. MRI has perfect contrast resolution and it is superior to US in demonstrating all the bony elements (femoral head, femoral neck and shaft, acetabulum, iliac bone) and cartilage together, in clearly depicting their relationship with each other, and in showing any impingement [8], regardless of any limiting factor experienced with US, such as adverse effects of tissue thickness and acoustic shadowing caused by bone.…”

Section: Discussionmentioning

confidence: 99%

Hip Ultrasonography in Classification of Proximal Focal Femoral Deficiency: Two Case Reports

Ayaz¹

2017

Ann Clin Anal Med

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ÖzetProksimal fokal femur yetmezliği (PFFY) bulunan iki infantta femur başlarını sonografik olarak göstermeyi ve ultrasonografi (US) katkısı ile PFFY sınıflaması yapmayı amaçladık. Sağ uyluğunda kısalık bulunan yedi aylık erkek olgunun (Olgu 1) radyografisinde sağ femurun proksimal yarısında belirgin hipoplazi saptandı. Aynı taraf asetabulum displazikti. US'de femur başı hipoplazikti. Sağ uyluğunda kısa-lık bulunan 16 günlük erkek olgunun (Olgu 2) radyografisinde sağ femurun sola kı-yasla daha kısa ve ince olduğu görüldü. Aynı taraf asetabulumun gelişimi yeterliydi. US'de femur başı normaldi. US, PFFY'li infantlarda kıkırdak femur başlarının durumunu göstererek Olgu 1 ve Olgu 2'nin, sırasıyla Aitken sınıf C ve A olarak sı-nıflandırılmasına yardımcı olmuştur. US, radyografi ile birlikte PFFY sınıflamasın-da ve diğer kalça eklemini değerlendirmede kullanılabilir. Anahtar KelimelerSüt Çocuğu; Ultrasonografi; Kalça Eklemi; Femur; Alt Ekstremite Deformiteleri, Doğumsal AbstractOur purpose was to sonographically demonstrate the femoral heads of two infants with proximal focal femoral deficiency (PFFD) and to classify PFFD with the contribution of ultrasonography (US). On the radiograph of Case 1, a sevenmonth-old male with a short right thigh, there was marked hypoplasia of the proximal half of the right femur. Ipsilateral acetabulum was dysplastic. On the US, the femoral head was hypoplastic. On the radiograph of Case 2, a 16-day-old male with a short right thigh, the right femur was shorter and thinner than the left femur. An adequate ipsilateral acetabulum was found. US revealed a normal femoral head. US helped the classification of Case 1 as Aitken class C and Case 2 as and Aitken class A, by demonstrating the status of cartilaginous femoral heads in PFFD. Combined with radiography, US can be used in the classification of PFFD and in the evaluation of the unaffected hip.

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“…3,9,10 MRI should be considered the modality of choice when assessing PFFD because of its ability to demonstrate the anatomy of the cartilaginous and bony proximal femur, which can facilitate appropriate treatment. 3,4,11 Osteo-articular infections in neonates are rare and usually present in an atypical way compared to older children. 12 Wilson et al 12 showed that infants can often have a worse outcome compared to older patients in their series possibly due to a delay in diagnosis.…”

Section: Discussionmentioning

confidence: 99%