A. Congenital Radioulnar Synostosis: Self-Directed Learning

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CHIR12007

Clinical Assessment and Diagnosis


Portfolio Exercises Week 8

Self-directed learning:

The following conditions are congenital disorders or alterations in development that may
have clinical consequences and may mimic other conditions.
Investigate the following conditions. Include a description of the condition, relevant clinical
history, any associated clinical findings, associated clinical or systemic features and any
additional information you feel may be important as a chiropractor. (also incl reference(s))

A. Congenital Radioulnar Synostosis

• Description:

- Congenital radioulnar synostosis is a rare disorder


resulting in the fusion of the radius and ulna from birth.
- Refers to the failure of segmentation of the adjacent
radius and ulna.
- Caused by the failure of separation of the fused
cartilaginous precursors of the radius and ulna during
the 7th week of gestation.

• Associated clinical findings, associated clinical or systemic features:

- Affects supination and pronation movements of the elbow joint (flexion and extension
of unaffected).
- Potentially creates excessive strain on the shoulder and wrist joint.
- CRS may be associated with other congenital musculoskeletal disorders such as
congenital hip dislocation, clubfeet, polydactyly, or syndactyly.
- Surgery to regain rotatory movement rarely succeeds.
- Poor surgical outcomes occur, because of the poorly developed supporting
- structures, neurovascular compromise, and recurrence of the synostosis.

• Addition information for the chiropractor:

- Over half of cases are bilateral.


- If the deformity is mild, the patient often compensates well with adjusted movements of
the adjacent joints, wrists, and shoulders.
- To avoid excessive strain on other joints, occupational therapy, activity modification, and
ergonomic working environment are recommended.

Reference:

Tsai, J. (2017). Congenital radioulnar synostosis. Radiology Case Reports, 12(3), 552-554. doi:
10.1016/j.rad cr.2017.03.011

B. Ulna Minus Variant

• Description:

- Ulnar variance (also known as Hulten variance) refers to the


relative lengths of the distal articular surfaces of the radius and
ulna.
- Ulna variance may be either; positive (ulna projects more
distally) or negative (ulna projects more proximally).
- Negative ulnar variance describes a state where the ulna is
abnormally shortened compared to the radius and plays an
important role in wrist pathology.

• Associated clinical findings, associated clinical or systemic features:

- Can be caused by trauma or mechanical issues (fracture or surgical shortening), it may


also be congenital (Madelung deformity).
- negative ulnar variance is associated with Kienbock's disease.
- Patients with negative ulnar variance and histories suggestive of ligamentous instability
should undergo careful radiologic evaluation to assure early diagnosis of carpal
disruption.

• Addition information for the chiropractor:

- To determine ulnar variance on radiographs, the generally accepted standard view is a


posteroanterior view obtained with the wrist in neutral forearm rotation, the elbow
flexed 90° and the shoulder abducted 90°.
- Negative variance is when the ulna is ≤2.5 mm than the radius at the DRUJ.
- a 2.5 mm decrease in the ulno-carpal variance will decrease the load borne by the ulno-
carpal joint to 4.3%.
Reference:

Radiopaedia. (2018). Ulnar Variance. Retrieved from https://radiopaedia.org/articles/ulnar-variance

C. Madelung’s Deformity

• Description:

- Madelung deformity is due to early closure of the medial


aspect of the distal epiphysis of the radius.
- Madelung deformity of the wrist is caused by abnormal
growth disturbance in the inferior volar part of the
epiphysial growth plate in the distal radius, resulting in a an
increase in the curvature of the distal radius that results in a
palmer subluxation of the hand in relation to the distal
radioulnar joint. The ulna continues growing straight,
resulting in a dorsally prominent distal ulna.

• Associated clinical findings, associated clinical or systemic features:

- Madelung's deformity of the wrist is a rare condition, usually bilateral, developing at the
distal end of the forearm of young individuals between the ages of 8 and 20 years.
- In females more than males.
- The lesion progresses slowly and by the end of one or two years the deformity is
complete.

- could be idiopathic or due to trauma.


- During the active developmental stage, pains may or may not be present.

• Addition information for the chiropractor:

- The deformity is characterized by a prominent dorsal subluxation of the ulnar head and
palmar sag of the hand and wrist with a shortened forearm.
- Can usually be viewed exteriorly (without x-ray).
- Stiffness, pain and extensor tendon ruptures have been reported.
- Conservative management is the gold standard for asymptomatic patients.
- Surgical management is reserved for cosmetic reasons or symptomatic patients.
- The deformity can be treated surgically by addressing the deforming bony and
ligamentous lesions, correcting the abnormal position of the radial articular surface, and
equalizing the longitudinal levels of the distal radius and ulna.

Reference:
Ghatan, A. C., & Hanel, D. P. (2013). Madelung Deformity. Journal of the American Academy of
Orthopaedic Surgeons, 21(6), 372-382.
http://dx.doi.org.ezproxy.cqu.edu.au/10.5435/JAAOS-21-06-372

D. Syndactyly

• Description:

- Syndactyly is the most common, inherited and clinically


heterogeneous congenital malformation of the limbs,
where there is a failure of differentiation in which the
fingers or toes fail to separate into individual
appendages.
- The term syndactyly is derived from the Greek prefix syn- ("with, together") and the
Greek noun daktylos ("finger, digit").

• Associated clinical findings, associated clinical or systemic features:

- Syndactyly is a failure in the separation of developing digits during organogenesis. This


separation usually occurs during the sixth and eighth weeks of embryologic
development.
- More common in males (2x)
- Commonly involves interspaces between middle and ring finger, ring and little finger
(can also happen between thumb and index or index and middle).

Syndactyly can be simple or complex.

- In simple syndactyly, adjacent fingers or toes are joined by soft tissue.


- In complex syndactyly, the bones of adjacent digits are fused.

Syndactyly can be complete or incomplete.


- In complete syndactyly, the skin is joined all the way to the tip of the involved digits.
- In incomplete syndactyly, the skin is only joined part of the distance to the tip of the
involved digits.

• Addition information for the chiropractor:

- The treatment goal for syndactyly is to create a more normal


web space to improve the function and appearance of each
finger.
- Management is still by surgical correction, and requires optimal planning in terms of
patient age and degree of malformation present. This is a non-urgent procedure and it
must take into account the psychological and social impact on the child.

- Despite increasing knowledge of the causes of syndactyly, management has not changed
greatly over the years, although the future is likely to see new techniques involving gene
manipulation and tissue engineering at least to be created and studied, although current
management is low risk.

Reference:
Braun, T., Trost, J., & Pederson, W. (2016). Paediatric Plastic Surgery Procedures. Syndactyly
Release. 30(4), 162-170. DOI: 10.1055/s-0036-1593478

E. Acro-osteolysis

• Description:

- This disease is the slow, progressive bone


reabsorption (osteolysis) of the most distal part
of a distal phalanx of the feet and hand and is
associated with a heterogeneous group of
pathological entities.
-
• Associated clinical findings, associated clinical or systemic features:

- Acro-osteolysis has two patterns of resorption: transverse and longitudinal.


- In the transverse type, linear bands of resorption are evident in the shaft of the distal
phalanx, perpendicular to its long axis.
- In the longitudinal type, there is resorption of the tuft of the distal phalanx.

• Addition information for the chiropractor:

- Nail changes may provide a useful diagnostic clue to


underlying bone resorption.
- Plain radiography is the gold standard for the
detection of acro-osteolysis.

Reference:

Botou, A., Bangeas, A., Alexiou, I., & Sakkas, L. (2017). Acro-osteolysis. Clinical Rheumatology, 36(1),
9-14. https://doi-org.ezproxy.cqu.edu.au/10.1007/s10067-016-3459-7

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