Case submitted by Dr. Ibrahim Tabsh

Introduction

This is a 3 year old boy who is diagnosed to have a skeletal dysplasia of an indeterminate nature. After an extensive workup by paediatricians, paediatric endocrinologists, and paediatric radiologists his case was presented to world leading specialists during several international meetings.  Their diagnosis was Short Limb Dwarfism.  His presenting problem was a limp.
 
Physical examination revealed dysmorhic facial features, and microcephaly.  His weight was 3 kg and he was below the third percentile for height.  However, his cognitive development was appropriate for a 3 year old boy, and he was able to walks and talk at this level.
 
Upper extremities: normal
Spine: mild thoracolumbar kyphosis, no scoliosis, but lumbar hyperlordosis
Hips: adductor tightness, mild fixed flexion contractures, and subluxed hips
Remainder of lower extremities: Normal alignment.

Radiographs
 

Describe the plain film findings.
 
What are the options for this boy ?

Plain films show bilateral acetabular dysplasia, significant subluxation Left > Right, on weight bearing views.  There is bilateral coxa vara, with increased femoral neck anteversion.

Options were discussed with the family.  These basically are: doing nothing vs. concentric open hip reduction.  The problem is that the long term prognosis in this disease is not known since the cause of the skeletal dysplasia is yet to be determined.   They elected for a surgical option.  

What surgical procedures would you recommend ?
Are there any modalities of treatment you would try before surgery ?
Would you recommend all surgery to be done at one time, or a staged procedure ?

A staged procedure was carried out with the RIGHT side first

Skin traction was used on both hips for ten days, and then the patient underwent:

1. EUA with Image intensifier
2. Bilateral Percutaneous Adductor Tenotomy
3. Right Salter Innominate Osteotomy (3 fully threaded Steinman Pins)
4. Single leg Hip spica casting for 6 weeks.

 

Postoperative Radiographs (Stage 1)

3 to 4 months later he underwent Stage 2 for the LEFT side:
1. EUA with image intensifier
2. Removal of Steinman Pins from right Ilium
3. Left Salter Innominate Osteotomy (2 fully threaded Steinman Pins)
4. Single leg Hip spica cast for 6 weeks

Intraoperative Photographs (Stage 2)

   

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