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Teapot syndrome with grade 4 acrofemoral synostosis

Case contributed by: Dr Matt Skalski

Presentation:

Known teapot syndrome.

Patient Data:

Age: 2

  

Case Discussion:

Typical case of teapot syndrome in a 2 year old characterized by right acrofemoral synostosis (the handle) and contralateral left radial ray anomaly (the spout). This congenital condition was universally fatal prior to the advent of caesarian section. 

Acrofemoral synostosis (AFS) is graded 1–5 corresponding to the number of digits fused to the femur and is invariably associated with developmental dysplasia of the hip. Historical attempts at surgical correction of AFS were unfavourable, as the severity of hip dysplasia interfered with mobility. In contrast, children with uncorrected AFS mobilize relatively freely by actively moving the leg with the attached upper limb in what has been termed the puppeteer adaptation. 

Interestingly, the song “I’m a little Teapot” created by George Harold Sanders and Clarence Z. Kelley in 1939 was inspired by a child with teapot syndrome. Sanders famously wrote the song to help include a boy, Handinma Pokett, in an urban jazz routine. Remarkably, Pokett went on to join the Bolshoi ballet and several of his most memorable performances are featured in this YouTube video.      

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