Al-saraf Ali,  Ong Seng

Final Pr. ID: Poster #: EDU-067

In the second part of this exhibit, congenital bone anomalies of the lower extremity will be identified and reviewed. Through this exhibit, the reader will be able to: 1. Define the most common entities encompassing congenital lower extremity bone anomalies based on classic clinical presentation and demographics. 2. Highlight key multi-modality imaging characteristics to accurately differentiate between the various congenital bone anomalies of the lower extremity. Read More

Authors:  Al-saraf Ali , Ong Seng

Keywords:  Tibial Hemimelia, Proximal Focal Femoral Deficiency, Amniotic Band Syndrome, Fibular Hemimelia, Tarsal Coalition

Marie Eman,  Perez Manuela,  Aquino Michael,  Stimec Jennifer

Final Pr. ID: Poster #: EDU-060

Although rare, fibular hemimelia or congenital fibular deficiency (CFD), is the most common congenital long bone deficiency, with an approximate incidence of 7.4- 20 per 1 million live births. The clinical presentation of CFD represents a broad spectrum of manifestations, ranging from mild fibular deficiency with limb length discrepancy to a significantly short limb with multiple associated foot, ankle and knee deformities.
Traditional FH classification such as Achterman and Kalamchi described the amount of fibular deficiency, which is today known to be unrelated to length discrepancy and foot deformity. Current classifications are based on the associated deformities of the ankle and subtalar joint, as the foot deformity is the main prognostic factor.
Treatment should be tailored for each patient to maximize the lower limb function - this involves predicting the limb length discrepancy and then coming up with a surgical plan to correct these in the fewest number of surgeries spread out as much as possible throughout the child’s growing years, so that by skeletal maturity the child has achieved equal leg length, a functional plantigrade foot, excellent alignment of the hip, knee and ankle and, as needed, a stable knee joint.
Multimodal imaging provides detailed evaluation of the osteochondral and extraosseous malformations. In-utero identification can be accomplished with prenatal ultrasonography. After birth, radiographs often show striking bony anomalies. Detailed information regarding associated crucial cartilaginous, articular, soft tissue, and vascular abnormalities required for preoperative planning necessitates the use of magnetic resonance (MR) imaging.
The purpose of this poster is to: 1) review the various types of osteochondral and extra-osseous abnormalities of CFD as depicted by different imaging modalities, 2) describe the limitations of each of these modalities, 3) outline the image-based classification of CFD, 4) describe the options for treatment, and 5) discuss the post-operative imaging evaluation of CFD. Read More

Authors:  Marie Eman , Perez Manuela , Aquino Michael , Stimec Jennifer

Keywords:  hemimelia, congenital fibular deficiency

Gokli Ami,  Koshy June,  Neuman Jeremy

Final Pr. ID: Poster #: EDU-001

Ulnar hemimelia is a rare congenital disorder characterized by absence of the ulna with an estimated incidence of 1/100,000-150,000 live births. The etiology of this disease remains uncertain, however it is thought that the deformity develops between the 4th and 7th week of gestation. The disease can be either partial or complete, and can be found in isolation or in association with other skeletal dysplasias such as Gorlin-Goltz Syndrome, Cornelia de Lange syndrome, Klippel-Feil syndrome, finger anomalies including syndactyly and oligodactyly. The purpose of this educational review is to describe the characteristic imaging findings of ulnar hemimelia with attention paid to clinically important associated features; review the differential diagnosis for ulnar anomalies; and discuss the range of congenital anomalies that can be associated with this condition. We will also perform a brief review of the literature concerning this rare entity, present two cases of complete ulnar hemimelia with oligodactyly from our institution, and review the various treatment options. Read More

Authors:  Gokli Ami , Koshy June , Neuman Jeremy

Keywords:  Hemimelia, Congenital, Pediatric