Proximal focal femoral deficiency: evaluation by MR imaging

Biko, David M.; Davidson, Richard S.; Pena, Andres; Jaramillo, Diego

doi:10.1007/s00247-011-2203-3

Cited by 32 publications

(26 citation statements)

References 13 publications

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“…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. However MRI is more costly than US and sedation is required in infants.…”

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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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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“…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%

“…1 Clinically patients typically present with a short and bulky thigh that is flexed, externally rotated and abducted. [1][2][3]6 A clinical paradox exists in that the 'hip joint' in PFFD is generally painless and relatively stable. 7,8 Westin and Gunderson 8 noted that 'hip pain is seldom a complaint' and that 'in spite of the complete separation of the shaft from the proximal element, little or no telescoping occurs with weight bearing'.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3] The clinical spectrum of PFFD is wide ranging from minimal shortening (femoral hypoplasia) in mild cases, to complete absence of the proximal femur and acetabulum in the most severe cases. 1 Clinically patients typically present with a short and bulky thigh that is flexed, externally rotated and abducted.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 PFFD occurs with an incidence of 1 in 52 000 live births. 3,4 This case report highlights the unusual presentation of double pathology of PFFD and associated osteomyelitis of the ipsilateral proximal femur with no preceding trauma or surgical intervention. This case shows how magnetic resonance imaging (MRI) can be useful in localising subperiosteal infections of the proximal femur in the presence of abnormal pre-existing anatomy in very young patients.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Osteomyelitis in an infant with proximal focal femoral deficiency

2016

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Embryology of the lower limb demonstrates that congenital absent fibula is a radiologic misnomer

Hootnick

Levinsohn

2021

The Anatomical Record

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The congenitally shortened limb (CSL) with fibular deficiency or absence has historically been graded by plain radiography, while associated cartilaginous and arterial soft tissue anomalies have been comparatively neglected. Consistent pathological evidence of remnant cartilaginous bodies in place of the fibula presupposes earlier existence of a preformed cartilaginous template of the fibula. In complete fibular radiographic absences, often associated with midline metatarsal deficiencies, the two usual nutrient arteries to the fibula fail to form, as they normally would have, around the (16-18 mm stage) sixth embryonic week. The histopathology of fallow persisting fibular anlagen, in association with missing arteries and retained primitive arteries, suggests the anlage is a dystrophic, but otherwise normally prefigured, cartilaginous scaffold of the fibula. Thus, the widely employed term absent fibula, which has been grounded in plain radiography, is a misnomer. Additionally, since the metatarsals missing in congenitally shortened limb are midline, the related term, fibular hemimelia, is similarly inaccurate. A new taxonomy, based on embryological principles rather than radiographic appearance alone, will promote limb dystrophism as a more accurate term combining arrested embryonic vascular development and congenitally shortened limb of the lower extremity.

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Proximal focal femoral deficiency: evaluation by MR imaging

Abstract: MRI can help in evaluation of PFFD by defining the anatomy. MR demonstrates features of the acetabulum and cartilaginous femoral epiphysis and depicts ligamentous abnormalities of the knee.

Cited by 32 publications

References 13 publications

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

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

Osteomyelitis in an infant with proximal focal femoral deficiency

Embryology of the lower limb demonstrates that congenital absent fibula is a radiologic misnomer

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