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Orthogate  Cases Pediatric Short Stature Dysmorphism DDH
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Short Stature Dysmorphism DDH |
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- Case submitted by Dr.
Ibrahim Tabsh
Click on images to view larger radiographs and clinical pictures
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:
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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.
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- Postoperative Radiographs (Stage 1)
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- 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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